Publications of the Wolfrum lab | Molecular Cell Biology

Peer-reviewed original publications:

- manuscripts submitted or under revision -

Tebbe L, Yildirim A, Sorusch N, Petit C, Wunderlich K, El-Amraoui A, Wolfrum U (20XY) Binding of Usher syndrome 1G protein SANS to microtubule depolymerizing kinesin KIF2A controls primary cilia length and maintenance (submitted)

Nießner C, Reinhard K, Wolfrum U, Meimann S, Winklhofer M, Peichl L (20XY) Potential magnetoreceptor cryptochrome 1 found in human retinal blue cone photoreceptors (submitted Sci Rep)

Kusuluri DK, Linnert J, Güler BE, Boldt K, Krzysko J, Ueffing M, Wolfrum U (202X) The BBS/CCT chaperonin complex determines the localization of the adhesion GPCR VLGR1/ADGRV1 (USH2C) to primary cilia (submitted)

Fritze JS, Stiehler FF, Wolfrum U (20XY) Pathogenic variants in USH1G/SANS alter the protein interaction with the pre-RNA processing factors PRPF6 and PRPF31 of the spliceosome (submitted)

- preprints and not certified by peer review -

Nagel-Wolfrum K, Fadl BR, Becker MM, Wunderlich KA, Schäfer J, Sturm D, Gür B, Kaplan L, Goldmann T, Brooks M, Starosk MR, Lokhande A, Apel M, Fath KR, Stingl K, Kohl S, Andrade M, Vetter JM, Pfeiffer N, Grosche A, Swaroop A, Wolfrum U (2021) Characterization of USH1C/harmonin in the human retina provides insights into pathophysiology and therapy options for Usher syndrome. bioRxiv doi: https://doi.org/10.1101/2021.08.27.457962

Grotz S, Schäfer J, Wunderlich KA, Ellederova Z, Auch H, Bähr A, Runa-Vochozkova P, Plutniok J, Arnold V, Ardan T, Veith M, Santamaria G, Dhom G, Hitzl W, Kessler B, Kurome M, Zakharchenko V, Linnert J, Fischer A, Blutke A, Döring A, Suchankova S, Popelar J, May-Simera H, Laugwitz KL, Vandenberghe LH, Wolf E, Nagel-Wolfrum K, Motlik J, Fischer MD, Wolfrum U*, Klymiuk N* (2021) Early Disruption of Photoreceptor Cell Architecture and Loss of Vision in a Humanized Pig Model of Usher Syndrome. bioRxiv 2021.05.31.446123: doi: https://doi.org/10.1101/2021.05.31.446123

Nagel-Wolfrum K, Fadl BR, Becker MM, Wunderlich KA, Schäfer J, Sturm D, Gür B, Kaplan L, Goldmann T, Brooks M, Starosk MR, Lokhande A, Apel M, Fath KR, Stingl K, Kohl S, Andrade M, Vetter JM, Pfeiffer N, Grosche A, Swaroop A, Wolfrum U (2021) Characterization of USH1C/harmonin in the human retina provides insights into pathophysiology and therapy options for Usher syndrome. bioRxiv doi: https://doi.org/10.1101/2021.08.27.457962.

- accepted & published -

Sanjurjo-Soriano C, Jimenez-Medina C, Erkilic N, Cappellino L, Lefevre A, Nagel-Wolfrum K, Wolfrum U, Van Wijk E, Roux AF, Meunier I, Kalatzis V (2023) USH2A variants causing retinitis pigmentosa or Usher syndrome provoke differential retinal phenotypes in disease-specific organoids. HGG Adv, 4:100229. doi: 10.1016/j.xhgg.2023.100229. PMID: 37654703; PMCID: PMC10465966.

187 Linnert J, Knapp B, Güler BE, Boldt K, Ueffing M, Wolfrum U (2023) Usher syndrome proteins ADGRV1/VLGR1 (USH2C) and CIB2 (USH1J) interact and share a common interactome containing CCT/BBS chaperonin complex molecules. Front Cell Dev Biol, 11:1199069. PMID: 37427378; PMCID: PMC10323441 doi: 10.3389/fcell.2023.1199069

186 Araç-Ozkan D, Aust G, Bonner TI, Cappallo-Obermann H, Formstone C, Hamann J, Harty B, Heyne H, Kirchhoff C, Knapp B, Krishnan A, Langenhan T, Le Duc D, Lin HH, Martinelli DC, Monk K, Piao X, Prömel S, Schöneberg T, Schiöth H, Singer K, Stacey M, Ushkaryov Y, Vallon M, Wolfrum U, Xu L (2023) Adhesion Class GPCRs in GtoPdb v.2023.1, IUPHAR/BPS Guide to Pharmacology CITE, https://doi.org/10.2218/gtopdb/F17/2023.1

185 Linnert J, Güler BE, Krzysko J, Wolfrum U (2023) The adhesion GPCR VLGR1/ ADGRV1 controls autophagy. Basic Clin Pharmacol Toxicol 2023 Apr 1. doi: 10.1111/bcpt.13869. Online ahead of print.

184 Güler BE, Linnert J, Wolfrum U (2023) Monitoring paxillin in astrocytes reveals the significance of the adhesion GPCR VLGR1/ADGRV1 for focal adhesion assembly. Basic Clin Pharmacol Toxicol. 2023 Mar 16. doi: 10.1111/bcpt.13860. Online ahead of print.

Schäfer J, Wenck N, Janik K, Linnert, J, Stingl, K, Kohl S, Nagel-Wolfrum K, Wolfrum U (2023) The Usher syndrome 1C protein harmonin regulates canonical Wnt signaling. (accepted by Front Cell Dev Biol. Jan 23rd, 2023)

Winkler R, Quaas M, Glasmacher S, Wolfrum U, Thalheim T, Galle J, Krohn K, Magin TM and Aust G (2022) The adhesion G protein-coupled receptor GPR115/ADGRF4 regulates epidermal differentiation and associates with cytoskeletal KRT1. Cells 2022, 11 (19), 3151. https://doi.org/10.3390/cells11193151

Krzysko J, Maciag F, Mertens A, Güler BE, Linnert J, Boldt K, Ueffing M, Nagel-Wolfrum K, Heine M, Wolfrum U (2022) The adhesion GPCR VLGR1/ADGRV1 regulates the Ca2+ homeostasis at mitochondria-associated ER membranes. Cells 2022, 11(18), 2790; https://doi.org/10.3390/cells11182790

Nagel-Wolfrum K, Fadl BR, Becker MM, Wunderlich KA, Schäfer J, Sturm D, Fritze J, Gür B, Kaplan L, Goldmann T, Brooks M, Starosk MR, Lokhande A, Apel M, Fath KR, Stingl K, Kohl S, DeAngelis MM, Schlötzer-Schrehardt U, Kim IK, Owen LA, Vetter JM, Pfeiffer N, Andrade-Navarro MA, Grosche A, Swaroop A, Wolfrum U (2022) Expression and subcellular localization of USH1C/harmonin in human retina provides insights into pathomechanisms and therapy. Hum Mol Genet ddac211, https://doi.org/10.1093/hmg/ddac211

Knapp B, Roedig J, Roedig H, Krzysko J, Horn N, Güler BE, Kusuluri DK, Yildirim A, Boldt K, Ueffing M, Liebscher I, Wolfrum U (2022) Affinity Proteomics Identifies Interaction Partners and Defines Novel Insights into the Function of the Adhesion GPCR VLGR1/ADGRV1. Molecules 2022, 27, 3108. https://doi.org/10.3390/molecules27103108

Grotz S, Schäfer J, Wunderlich KA, Ellederova Z, Auch H, Bähr A, Runa-Vochozkova P, Fadl J, Arnold V, Ardan T, Veith M, Santamaria G, Dhom G, Hitzl W, Kessler B, Kurome M, Zakharchenko V, Linnert J, Fischer A, Blutke A, Döring A, Suchankova S, Popelar J, Rodríguez-Bocanegra E, Peters T, Dlugaiczyk J, Straka H, May-Simera H, Laugwitz KL, Wang W, Vandenberghe LH, Wolf E, Nagel-Wolfrum K, Motlik J, Fischer MD, Wolfrum U*, Klymiuk N* (2022) Early disruption of photoreceptor cell architecture and loss of vision in a humanized pig model of Usher syndrome. EMBO Mol Med, accepted Feb. 9th 2022 DOI: 10.15252/emmm.202114817.

Turn ER, Hu Y, Deweesa SI, Devia N, Easte MP, Hardin KR, Khatib T, Linnert J, Wolfrum U, Limi MJ, Casanova EJ, Caspary T, and Kahn RK (2021) The ARF GAPs ELMOD1 and ELMOD3 act at the Golgi and cilia to regulate ciliogenesis and ciliary protein traffic. Mol Biol Cell, 33(2):ar13, doi: 10.1091/mbc.E21-09-0443. accepted Nov 19, Epub 2021 Nov 24. PMID: 34818063.

Güler BE, Krzysko J, Wolfrum U (2021) Isolation and culturing of primary mouse astrocytes for the analysis of focal adhesion dynamics. STAR protocols, 2(4), 100954. doi.org/10.1016/j.xpro.2021.100954. eCollection 2021 Dec 17. PMID: 34917973.

Yildirim A, Mozaffari-Jovin S, Wallisch, A-K, Schäfer J, Ludwig SEJ, Urlaub H, Lührmann R and Wolfrum U (2021) SANS (USH1G) regulates pre-mRNA splicing by mediating the intra-nuclear transfer of tri-snRNP complexes. Nucleic Acids Res. 49(10):5845-5866. doi: 10.1093/nar/gkab386. PMID: 34023904.

Kusuluri DK, Güler, BE, Knapp B, Horn N, Boldt K, Ueffing M, Aust G, Wolfrum U (2021) The adhesion GPCR VLGR1 regulates cell migration by sensing mechanical cues at focal adhesions. iScience 24(4):102283. doi: 10.1016/j.isci.2021.102283. eCollection 2021 Apr 23. PMID: 33851099.

Turn ER, Linnert J, Gigante ED, Wolfrum U, Caspary T, and Kahn RA (2021) Roles for ELMOD2 and rootletin in ciliogenesis. Mol Biol Cell 32(8):800-822. doi: 10.1091/mbc.E20-10-0635. Epub 2021 Feb 17.PMID: 33596093.

Beryozkin A, Matsevich C, Obolensky A, Kostic C, Arsenijevic Y, Wolfrum U, Banin E, Sharon D (2020) A new mouse model for retinal degeneration due to Fam161a deficiency. Sci Rep11(1):2030. doi: 10.1038/s41598-021-81414-1171 doi: 10.1038/s41598-021-81414-1.PMID: 33479377 .

Corral-Serrano JC, van Reeuwijk J, Lamers IJC, Duijkers L, Hoogendoorn ADM, Yildirim A, Ruigrok RAA, Letteboer SJF, Butcher R, van Essen MD, Sakami S, van Beersum SEC, Palczewski K, Liu Q, Boldt K, Wolfrum U, Ueffing M, Garanto A, Roepmam R, Collin RWJ (2020) The photoreceptor-specific protein PCARE interacts with WASF3 to deploy a ciliary actin dynamics module. Proc Natl Acad Sci U S A 117(18):9922-9931 PMID: 32312818 PMCID: PMC7211956 DOI: 10.1073/pnas.1903125117

Zimmermann C, Krämer N, Krauter S, Strand D, Sehn E, Wolfrum U, Freiwald A, Butter F, Plachter B (2020) Autophagy as an antiviral defense mechanism is subverted by human cytomegalovirus. Autophagy. 17(3):779-795. doi: 10.1080/15548627.2020.1732686. Epub 2020 Mar 1.PMID: 32079454.

Reinehr S, Buschhorn V, Maliha AM, Goldmann T, Grus FH, Wolfrum U, Dick HB, Joachim SC (2020) Occurrence of retinal ganglion cell loss via autophagy and apoptotic pathways in an autoimmune glaucoma model. Curr Eye Res. 45(9):1124-1135. doi: 10.1080/02713683.2020.1716987. Epub 2020 Feb 3.PMID: 31935132.

Nagel-Wolfrum K, Buschhorn V, Wolfrum U, Stingl K (2019) Usher-Syndrom - Diagnose, genetische Ursachen, molekulare Mechanismen und potentielle Therapiemöglichkeiten. Z. prakt. Augenheilkd. 40: 487–499.

Tabujew I, Heidari M, Freidel C, Helm M, Tebbe L, Wolfrum U, Nagel-Wolfrum K, Koynov K, Biehl P, Schacher F, Potestio R, Peneva K (2019) Tackling the limitations of copolymeric siRNA delivery agents by a combined experimental-computational approach. Biomacromolecules, accepted Oct. 15.

Allon G, Mann I, Remez L, Sehn E, Rizel L, Nevet MJ, Perlman I, Wolfrum U, Ben-Yosef, T (2019) PRCD is Concentrated at the Base of Photoreceptor Outer Segments and is Involved in Outer Segment Disc Formation. Hum Mol Genet. 28(24):4078-4088. doi: 10.1093/hmg/ddz248 PMID: 31628458.

Sorusch N, Yildirim, A, Knapp B, Janson J, Fleck W, Scharf C, and Wolfrum U (2019) SANS (USH1G) molecularly links the human Usher syndrome protein network to the intraflagellar transport module by direct binding to IFT-B proteins. Front Cell Dev Biol. 2019 Oct 4; 7:216. doi: 10.3389/fcell.2019.00216. eCollection 2019. PMID: 31637240.

Knapp B, Roedig J, Boldt K, Krzysko J, Horn N, Ueffing M, Wolfrum U (2019) Affinity proteomics identify novel functional modules related to adhesion GPCRs. Ann N Y Acad Sci 1456:144-167. doi: 10.1111/nyas.14220. Epub 2019 Aug 22. PMID: 31441075.

Morgan RK, Anderson GR, Araç D, Aust G, Balenga N, Boucard A, Bridges JP, Engel FB, Formstone CJ, Glitsch MD, Gray RS, Hall RA, Hsiao CC, Kim HY, Knierim AB, Kusuluri DK, Leon K, Liebscher I, Piao X, Prömel S, Scholz N, Srivastava S, Thor D, Tolias KF, Ushkaryov YA, Vallon M, Van Meir EG, Vanhollebeke B, Wolfrum U, Wright KM, Monk KR, Mogha A (2019) The expanding functional roles and signaling mechanisms of adhesion G protein–coupled receptors. Ann N Y Acad Sci. 2019 Jun 6. doi: 10.1111/nyas.14094. [Epub ahead of print] PMID: 31168816.

van Dam TJP, Kennedy J, van der Lee R, de Vrieze E, Wunderlich KA, Rix S, Dougherty GW, Lambacher NJ, Li C, Jensen VL, Leroux MR, Hjeij R, Horn N, Texier Y, Wissinger Y, van Reeuwijk J, Wheway G, Knapp B, Scheel JF, Franco B, Mans DA, van Wijk E, Képès F, Slaats GG, Toedt G, Kremer H, Omran H, Szymanska K, Koutroumpas K, Ueffing M, Nguyen TT, Letteboer SJF, Oud MM, van Beersum SEC, Schmidts M, Beales PL, Lu Q, Giles RH, Szklarczyk R, Russell RB, Gibson TJ, Johnson CA, Blacque OE, Wolfrum U, Boldt K, Roepman R, Hernandez-Hernandez V, Huynen MA. (2019) CiliaCarta: an integrated compendium of ciliary genes. PLoS One. 14(5):e0216705. doi: 0.1371/journal.pone.0216705. eCollection 2019. PMID: 31095607.

Chakraborty D, Felzen V, Hiebel C, Stürner E, Perumal N, Manicam C, Sehn E, Grus F, Wolfrum U, Behl C, (2019) Enhanced autophagic-lysosomal activity and increased BAG3-mediated selective macroautophagy as adaptive response of neuronal cells to chronic oxidative stress. Redox Biology 24 (2019) 101181.

Knapp B, Kusuluri DK, Horn N, Boldt K, Ueffing M, Wolfrum U (2018) Identification of Protein Complexes Associated with the Usher Syndrome 2C and Epilepsy-Associated Protein VLGR1 Applying Affinity Proteomics. Genomics and Computational Biology. Vol. 4, No 1 e100051.

Zimmermann C, Büscher N, Krauter S, Krämer N, Wolfrum U, Sehn E, Tenzer S, Plachter B (2018) The abundant tegument protein pUL25 of human cytomegalovirus prevents proteasomal degradation of pUL26 and supports its suppression of ISGylation. J Virol. 2018 Oct 3. pii: JVI.01180-18. doi: 10.1128/JVI.01180-18. [Epub ahead of print] PMID: 30282718.

Dona M, Slijkerman RWN, Lerner KM, Broekman S, Wegner J, Howat TH, Peters T, Hetterschijt L, Boon N, de Vrieze E, Sorusch N, Wolfrum U, Kremer H, Neuhauss SC, Zang J, Kamermans M, Westerfield M, Phillips JB and van Wijk E (2018) Usherin defects lead to early-onset retinal dysfunction in zebrafish. Exp Eye Res 173:148-159. doi: 10.1016/j.exer.2018.05.015. PMID: 29777677.

Wolfrum U and Nagel-Wolfrum K (2018) The Usher Syndrome, a Human Ciliopathy. Klin Monbl Augenheilkd. 235(3):273-280. PMID: 29534264.

May-Simera H, Nagel-Wolfrum K and Wolfrum U (2017) Cilia - the sensory antennae in the eye. Prog Retinal Eye Res. 60:144-180. PMID: 28785766.

Birtel J, Gliem M, Mangold E, Tebbe L, Spier I, Müller PL, Holz FG, Neuhaus C, Wolfrum U, Bolz HJ, Charbel Issa P (2017) Novel insights into the phenotypical spectrum of KIF11-associated retinopathy, including a new form of retinal ciliopathy. Invest Ophthalmol Vis Sci. 58:3950-3959. PMID: 28785766.

Sorusch N, Bauß K, Plutniok J, Samanta A, Knapp B, Nagel-Wolfrum K and Wolfrum U (2017) Characterization of the ternary Usher syndrome SANS/ush2a/whirlin protein complex Hum Mol Genet. 26(6):1157-1172. PMID: 28137943.

Jovancevic N, Wunderlich K, Haering C, Flegel C, Maßberg D, Weinrich M, Weber L, Kampik A, Gisselmann G, Wolfrum U, Hatt H, Gelis L (2017) Deep sequencing of the human retinae reveals the expression of odorant receptors in the eye. Front Cell Neurosci. 2017 Jan 24;11:03. doi: 10.3389/fncel.2017.00003. eCollection 2017. PMID: 28174521.

Boldt K, van Reeuwijk J, Lu Q, Koutroumpas K, Nguyen TM, Texier Y, van Beersum SE, Horn N, Willer JR, Mans DA, Dougherty G, Lamers IJ, Coene KL, Arts HH, Betts MJ3,4, Beyer T, Bolat E, Gloeckner CJ, Haidari K, Hetterschijt L, Iaconis D, Jenkins D, Klose F, Knapp B, Latour B, Letteboer SJ, Marcelis CL, Mitic D, Morleo M, Oud MM, Riemersma M, Rix S, Terhal PA, Toedt G, van Dam TJ, de Vrieze E, Wissinger Y, Wu KM, Apic G, Beales PL, Blacque OE, Gibson TJ, Huynen MA, Katsanis N, Kremer H, Omran H, van Wijk E, Wolfrum U, Kepes F, Davis EE, Franco B, Giles RH, Ueffing M, Russell RB, Roepman R (2016) An organelle-specific protein landscape identifies novel diseases and molecular mechanisms. Nat. Commun 7:11491. doi: 10.1038/ncomms11491. PMID: 27182469.

Knapp B and Wolfrum U (2016) Adhesion G protein-coupled receptor related protein networks. Handb Exp Pharmacol. 2016; 234:147-178. PMID: 27832488.

Wunderlich KA and Wolfrum U (2016) A FRAP-based method for monitoring ciliary transport in photoreceptor cells in vivo. In: S.T. Christensen, P. Satir (eds.): Cilia; Methods Mol Biol. 2016;1454:97-106. PMID: 27514918.

Nguyen PNO, Böhm S, Gießl A, Butz ES, Wolfrum U, Brandstätter JH, Wahl-Schott C, Biel M, Becirovic E (2016) Peripherin-2 differentially interacts with cone opsins in outer segments of cone photoreceptors. Hum Mol Genet 25(12):2367-2377. PMID: 27033727.

Dinculescu A, Stupay RM, Deng WT, Frank DM, Min SH, Boye SL, ChiodoVA, Abrahan CE, Zhu P, Li Q, Strettoi E, Novelli E, Nagel-Wolfrum K, Wolfrum U, Smith WC, Hauswirth WW (2016) AAV-mediated Clarin-1 expression in the mouse retina: Implications for USH3A gene therapy. PLoS One 11(2):e0148874. doi: 10.1371/journal.pone.0148874. eCollection 2016.

Krömmelbein N, Wiebusch L, Schiedner G, Büscher N, Sauer C, Florin L, Sehn E, Wolfrum U, Plachter B (2016) Adenovirus E1A/E1B transformed amniotic fluid cells support human cytomegalovirus replication. Viruses 2016 Feb 2; 8(2). PMID: 26848680.

Nagel-Wolfrum K, Möller F, Penner I, Baasov T, Wolfrum U (2016) Targeting nonsense mutations in diseases with translational read-through-inducing drugs (TRIDs). BioDrugs 30(2):49-74. doi: 10.1007/s40259-016-0157-6.PMID: 26886021.

Sanders AA, de Vrieze E, Alazami AM, Alzahrani F, Malarkey EB, Sorusch N, Tebbe L, Kuhns S, van Dam TJ, Alhashem A, Tabarki B, Lu Q, Lambacher NJ, Kennedy JE, Bowie RV, Hetterschijt L, van Beersum S, van Reeuwijk J, Boldt K, Kremer H, Kesterson RA, Monies D, Abouelhoda M, Roepman R, Huynen MH, Ueffing M, Russell RB, Wolfrum U, Yoder BK, van Wijk E, Alkuraya FS, Blacque OE (2015) KIAA0556 is a novel ciliary basal body component mutated in Joubert syndrome. Genome Biol 16(1):293. doi: 10.1186/s13059-015-0858-z PMID: 26714646.

Jansen F, Kalbe B, Scholz P, Mikosz M, Wunderlich KA, Kurtenbach S, Nagel-Wolfrum K, Wolfrum U, Hatt H, Osterloh S (2015) Impact of the Usher syndrome on olfaction. Hum Mol Genet 25:524-533 PMID: 26620972.

Zaki MS, Heller R, Thoenes M, Nürnberg G, Stern-Schneider G, Nürnberg P, Karnati S, Swan D, Fateen E, Nagel-Wolfrum K, Mostafa MI, Thiele H, Wolfrum U, Baumgart-Vogt E, Bolz HJ (2015) PEX6 is expressed in photoreceptor cilia and mutated in deafblindness with enamel dysplasia and microcephaly. Hum Mutat 37:170-4. doi: 10.1002/humu.22934. [Epub ahead of print] PMID: 26593283.

Cerny AC, Altendorfer a, Schopf K, Baltner K, Maag N, Sehn E, Wolfrum U, Huber A (2015) The GTP- and phospholipid-binding protein TTD14 regulates trafficking of the TRPL ion channel in Drosophila photoreceptor cells. PLoS Genet 2015 Oct 28; 11(10):e1005578. PMID: 26509977.

Kunst S, Wolloscheck T, Kelleher DK, Wolfrum U, Sargsyan SA, Iuvone PM, Baba K, Tosini G, Spessert R (2015) Pgc-1α and Nr4a1 Are Target Genes of Circadian Melatonin and Dopamine Release in Murine Retina. Invest Ophthalmol Vis Sci 56:6084-94 PMID: 26393668.

Stephen LA, Tawamie H, Davis GM, Tebbe L, Nürnberg P, Nürnberg G, Thiele H, Thoenes M, Boltshauser E, Uebe S, Rompel O, Reis A, Ekici AB, McTeir L, Fraser AM, Hall EA, Mill P, Daudet N, Cross C, Wolfrum U, Jamra RA, Davey MG, Bolz HJ (2015) TALPID3 controls centrosome and cell polarity and the human ortholog KIAA0586 is mutated in Joubert syndrome (JBTS23). Elife 2015 Sep 19;4. doi: 10.7554/eLife.08077. PMID: 26386247.

Khan AO, Eisenberger T, Nagel-Wolfrum K, Wolfrum U, Bolz HJ (2015) C21orf2 encodes a photoreceptor ciliary protein and is mutated in recessive early-onset retinal dystrophy with macular staphyloma. Br J Ophthalmol 2015 Aug 20. pii: bjophthalmol-2015-307277. doi: 10.1136/bjophthalmol-2015-307277. [Epub ahead of print] PMID: 26294103.

Wheway G, Schmidts M, Mans DA, Szymanska K, Nguyen TM, Racher H, Phelps IG, Toedt G, Kennedy J, Wunderlich KA, Sorusch N, Abdelhamed ZA, Natarajan S, Herridge W, van Reeuwijk J, Horn N, Boldt K, Parry DA, Letteboer SJF, Roosing S, Adams M, Bell SM, Bond J, Higgins J, Morrison EE, Tomlinson DC, Slaats GG, van Dam TJP, Huang L, Kessler K, Giessl A, Logan CV, Boyle EA, Shendure J, Anazi S, Aldahmesh M, Al Hazzaa S, Hegele RA, Ober C, Frosk P, Mhanni AA, Chodirker BN, Chudley AE, Lamont R, Bernier FP, Beaulieu CL, Gordon P, Pon RT, Donahue C, Barkovich AJ, Wolf L, Toomes C, Thiel CT, Boycott KM, McKibbin M, Inglehearn CF, UK10K Consortium, University of Washington Center for Mendelian Genomics, Stewart F, Omran H, Huynen MA, Sergouniotis PI, Alkuraya FS, Parboosingh JS, Innes AM, Willoughby CE, Giles RH, Webster AR, Ueffing M, Blacque O, Gleeson JG, Wolfrum U, Beales PL, Gibson T, Doherty D, Mitchison HM, Roepman R, Johnson CA (2015) An siRNA-based functional genomics screen for the identification of novel regulators of ciliogenesis and ciliopathy genes. Nat Cell Biol 17:1074-87. PMID: 26167768.

Marjanović M, Sánchez C, Terré B, Gomez R, Scheel J, Pacheco S, Knobel P, Martínez-Marchal A, Aivio S, Palenzuela L, Wolfrum U, McKinnon P, Suja J, Roig I, Costanzo V, Lüders J, Stracker T (2015) CEP63 deficiency promotes p53-dependent microcephaly and reveals a role for the centrosome in meiotic recombination. Nat Commun 2015 Jul 9;6:7676. PMID: 26158450.

Felzen V, Morawe T, Koziollek-Drechsler I, Reißig S, Koegel D, Wolfrum U, Brandts C, Behl C, Hiebel C (2015) Estrogen receptor α regulates non-canonical autophagy that provides stress resistance to neuroblastoma and breast cancer cells and involves BAG3 function. Cell Death Dis Jul 9;6:e1812. PMID: 26158518.

Palfi A, Chadderton N, O'Reilly M, Nagel-Wolfrum K, Wolfrum U, Bennett J, Humphries P, Kenna PF, Millington-Ward S, Farrar GJ (2015) Efficient gene delivery to photoreceptors using AAV2/rh10 and rescue of the Rho^-/- mouse. Mol Ther Methods Clin Dev 2:15016. PMID: 25989602.

Karam A, Tebbe L, Weber C, Messaddeq N, Morlé L, Kessler P, Wolfrum U, Trottier Y (2015) A Novel Function of Huntingtin in the Cilium and Retinal Ciliopathy in Huntington's Disease Mice Corresponding. Neurobiol Dis 80:15-28. PMID: 25989602.

Schubert T, Gleiser C, Heiduschka P, Franz C, Nagel-Wolfrum K, Sahaboglu A, Weisschuh N, Eske G, Rohbock K, Rieger N, Paquet-Durand F, Wissinger B, Wolfrum U, Hirt B, Singer W, Rüttiger L, Zimmermann U, Knipper M (2015) Deletion of myosin VI causes slow retinal optic neuropathy and age-related macular degeneration (AMD)-relevant retinal phenotype. Cell Mol Life Sci 72:3953-69. PMID: 25939269.

Hidalgo-de-Quintana J , Schwarz N, Porpino Meschede I, Stern-Schneider G, Powner M, Morrison E, Futter C, Cheetham ME, Wolfrum U, van der Spuy J (2015) The Leber congenital amaurosis protein AIPL1 associates with EB proteins at the photoreceptor cilium. PLoS One 2015 Mar 23; 10(3):e0121440. PMID: 25799540.

Hamann J, Aust G, Araç D, Engel FB, Formstone C, Fredriksson R, Harty BL, Kirchhoff C, Knapp B, Krishnan A, Liebscher I, Lin HH, Martinelli DC, Monk KR, Peeters MC, Piao X, Prömel S, Schöneberg T, Schwartz TW, Singer K, Stacey M, Ushkaryov YA, Vallon M, Wolfrum U, Wright MW, Xu L, Langenhan T, Schiöth HB (2015) International Union of Basic and Clinical Pharmacology. XC. Adhesion G protein-coupled receptors. Pharmacol Rev 67:338-67. PMID: 25713288.

Kunst S, Wolloscheck T, Grether M, Trunsch P, Wolfrum U, Spessert R (2015) Photoreceptor cells display a daily rhythm in the orphan receptor Esrrβ. Mol Vis 2015 Feb 19;21:173-84. eCollection 2015. PMID: 25737630.

Liebscher I, Ackley B, Araç D, Ariestanti DM, Aust G, Bae B, Bridges JB, Duman JG, Engel FB, Giera S, Goffinet A, Hall, R, Hamann J, Hartmann N, Lin HH, Liu M, Luo R, Mogha A, Monk KR, Peeters MC, Prömel S, Ressl S, Schiöth HB, Sigoillot MS, Song H, Talbot WS, Tall GG,White JP, Wolfrum U, Xu L, Langenhan T, Piao X (2014) New functions and signaling mechanisms for the class of adhesion G protein-coupled receptors. Ann N Y Acad Sci 1333:43-64. PMID: 25424900.

Schwarz N, Carr AJ, Lane A, Moeller F, Chen LL, Aguilà M, Muthiah MN, Davidson AE, Kanuga N, Wolfrum U, Nagel-Wolfrum K, Cruz L, Coffey PJ, Cheetham ME, Hardcastle AJ (2015) Translational read-through of the Retinitis pigmentosa 2 (RP2) Arg120stop mutation in patient iPSC-derived retinal pigment epithelial cells. Hum Mol Genet 24:972-86. PMID: 25292197.

Beck BB, Phillips JB, Bartram MP, Wegner J, Thoenes M, Pannes A, Sampson J, Heller R, Göbel H, Koerber F, Neugebauer A, Hedergott A, Nürnberg G, Nürnberg P, Thiele H, Altmüller J, Toliat MR, Staubach S, Boycott KM, Valente EM, Janecke AR, Eisenberger T, Bergmann C, Tebbe L, Wang Y, Wu Y, Fry AM, Westerfield M, Wolfrum U, Bolz HJ (2014) Mutation of POC1B in severe syndromic retinal ciliopathy. Hum Mut 35:1153-62. PMID: 25044745.

Becirovic E, Nguyen ON, Paparizos C, Butz ES, Stern-Schneider G, Wolfrum U, Hauck SM, Ueffing M, Wahl-Schott C, Michalakis S, Biel M (2014) Peripherin-2 couples rhodopsin to the CNG channel in outer segments of rod photoreceptors. Hum Mol Genet 23:5989-97. PMID: 24963162.

Nagel-Wolfrum K, Möller F, Penner I, Wolfrum U (2014) Translational read-through as an alternative approach for ocular gene therapy of retinal dystrophies caused by in-frame nonsense mutations. Vis Neurosci 10:1-8. PMID: 24912600.

Karlstetter M*, Sorusch N*, Caramoy A, Dannhausen K, Scheiffert E, Mirza M, Aslanidis A, Boesl M, Stoehr H, Nagel-Wolfrum K, Tamm ER, Jägle H, Wolfrum U^$, Langmann T^$ (2014) Disruption of the Retinitis Pigmentosa 28 gene Fam161a in mice affects the photoreceptor ciliary machinery and leads to retinal degeneration. Hum Mol Genet 23:5197-5210. PMID: 24833722 (* and ^$equal contributions).

Sergouniotis PI, Chakarova C, Murphy C, Becker M, Lenassi E, Arno G, Lek M, MacArthur DG, UCL-exomes consortium, Bhattacharya SS, Farooqi IS, Moore AT. Holder GE, Robson AG, Wolfrum U, Webster AR, Plagnol V (2014) Biallelic variants in TTLL5, encoding a tubulin glutamylase, cause retinal dystrophy. Am J Hum Genet 94:760-769. PMID: 24791901.

Sorusch N, Wunderlich K, Bauss K, Nagel-Wolfrum K, Wolfrum U (2014) Usher syndrome protein network functions in the retina and their relation to other retinal ciliopathies. Adv Exp Med Biol 801:527-533. PMID: 24664740.

Nagel-Wolfrum K, Baasov T, Wolfrum U (2014) Therapy strategies for Usher syndrome type 1C in the retina. Adv Exp Med Biol 801:741-774. PMID: 24664766.

Bauss K, Knapp B, Jores P, Roepman R, Kremer H, v. Wijk E, Maerker T, Wolfrum U (2014) Phosphorylation of the Usher syndrome protein SANS controls Magi2-mediated endocytosis Hum Mol Genet 23:3923-42. PMID: 24608321.

Borman AD, Pearce LR, Mackay DS, Nagel-Wolfrum K, Davidson AE, Henderson R, Garg S, Waseem NH, Webster AR, Plagnol V, Wolfrum U, Farooqi IS, Moore AT (2014) A Homozygous mutation in the TUB gene associated with retinal dystrophy and obesity. Human Mutation 35:289-93. PMID: 24375934.

van Dam TJP, Wheway G, Slaats GG, SYSCILIA Study Group, Huynen MA, Giles RH (2013) The SYSCILIA gold standard (SCGSv1) of known ciliary components and its applications within a systems biology consortium Cilia 2013, 2:7http://www.ciliajournal.com/content/2/1/7. PMID:23725226.

Smith TS, Spitzbarth B, Li J, Dugger DR, Stern-Schneider G, Sehn E, Bolch SN, McDowell JH Tipton J, Wolfrum U, Smith CW (2013) Light-Dependent Phosphorylation of Bardet Biedl Syndrome 5 in Photoreceptor Cells Modulates its Interaction with Arrestin1. Cell Mol Life Sci 70:4603-16. PMID: 23817741.

Davidson AD, Schwarz N, Zelinger L, Stern-Schneider G, ShoemarkA , Spitzbarth B, Gross M, Laxer U, Sosna J, Sergouniotis PI, Waseem NH, Wilson R, Kahn RA, Plagnol V , Wolfrum U, Banin E, Hardcastle AJ, Cheetham ME., Sharon D, Webster AR (2013) Mutations in ARL2BP, encoding ADP-ribosylation factor-like 2 binding protein, cause autosomal recessive retinitis pigmentosa. Am J Hum Genet 93:321-9. PMID: 23849777.

Nagel-Wolfrum K and Wolfrum U (2013) Vesicle transport and photoreceptor death: Fishing for molecular links. Dev Cell 25:435-6. PMID: 23763944.

Papal S, Cortese M, Legendre K, Sorusch N, Dragavon J, Sahly I, Shorte S, Wolfrum U, Petit C, El-Amraoui A (2013) The giant spectrin βV couples the molecular motors to phototransduction and Usher syndrome type I proteins along their trafficking route. Hum Mol Genet 22:3773-88. PMID: 23704327.

Avasthi P*, Scheel JF*, Ying G, Frederick JM, Baehr W, Wolfrum U (2013) Germline deletion of Cetn1 causes infertility in male mice. J Cell Sci 126:3204-13. PMID: 23641067 (*shared authorship).

Ghossoub R, Hu Q, Failler M, Rouyez MC, Spitzbarth B, Mostowy S, Wolfrum U, Saunier S, Cossart P, Nelson WJ, Benmerah A (2013) Septins 2, 7, and 9 and MAP4 co-localize along the axoneme in the primary cilium and control ciliary length. J Cell Sci 126:3204-13. PMID: 23572511.

Kunst S, Wolloscheck T, Hölter P, Wengert A, Grether M, Sticht C, Weyer V, Wolfrum U, Spessert R (2013) Transcriptional analysis of rat photoreceptor cells reveals daily regulation of genes important for visual signaling and light damage susceptibility. J Neurochem 124:757-69. PMID: 23145934.

Fischer DM, Goldmann T, Wallrapp C, Mühlfriedel R, Beck SC, Stern-Schneider G, Ueffing M, Wolfrum U, Seeliger MW (2013) Successful subretinal delivery and monitoring of MicroBeads in mice. PLoS One 8e55173. doi: 10.1371/journal.pone.0055173. PMID: 23383096.

Chadderton N, Palfi A, Millington-Ward S, Gobbo O, Overlack N, Carrigan M, O'Reilly M, Campbell M, Ehrhardt C, Wolfrum U, Humphries P, Kenna PF, Jane Farrar G (2013) Intravitreal delivery of AAV-NDI1 provides functional benefit in a murine model of Leber hereditary optic neuropathy. Eur J Hum Genet 21:62-8. PMID: 22669418.

Hölter P, Kunst S, Wolloscheck T, Kelleher DK, Sticht C, Wolfrum U, Spessert R (2012) The retinal clock drives the expression of Kcnv2, a channel essential for visual function and cone survival. Invest Ophthalmol Vis Sci 53: 6947-54. PMID: 22969075.

Jahn MR, Nawroth T, Fütterer S, Wolfrum U, Kolb U, Langguth P (2012) Iron oxide/hydroxide nanoparticles with negatively charged shells show increased uptake in Caco-2 cells. Mol Pharm 9:1628-37. PMID: 22587679.

Schladt TD, Koll K, Prüfer S, Bauer H, Natalio F, Dumele O, Raidoo R, Weber S, Wolfrum U, Schreiber LM, Radsak MP, Schild H, Tremel W (2012) Multifunctional superparamagnetic MnO@SiO2 core/shell nanoparticles and their application for optical and magnetic resonance imaging. J Mater Chem 22:9253-9262.

Araç D, Aust G, Calebiro D, Engel FB, Formstone C, Goffinet A, Hamann J, Kittel RJ, Liebscher I, Lin HH, Monk KR, Petrenko A, Piao X, Prömel S, Schiöth HB, Schwartz TW, Stacey M, Ushkaryov YA, Wobus M, Wolfrum U, Xu L, Langenhan T (2012) Dissecting signaling and functions of adhesion G protein-coupled receptors. Ann N Y Acad Sci 1276:1-25. PMID: 23215895.

Goldmann T, Overlack N, van Wyk M, Möller, Belakhov V, Baasov T, Wolfrum U*, Nagel-Wolfrum K* (2012) A comparative evaluation of NB30, NB54 and PTC124 in translational read-through efficacy for treatment of an USH1C nonsense mutation. EMBO Mol Med 4:1186-99. PMID: 23027640 (*equal contributions)

Zach F, Grassmann F, Langmann T, Sorusch N, Wolfrum U, Stöhr H (2012) The retinitis pigmentosa 28 protein FAM161A is a novel ciliary protein involved in intermolecular protein interaction and microtubule association. Hum Mol Genet 21:4573-86; 2012 Aug 23. PMID: 22791751.

Kersten F, van Wijk E, Hetterschijt L, Bauß K, Peters T, Aslanyan M, van der Zwaag B, Wolfrum U, Keunen J, Roepman R, Kremer H (2012) The mitotic spindle protein SPAG5/Astrin connects postmitotically to the Usher protein network. Cilia 1(1):2. doi: 10.1186/2046-2530-1-2. PMID: 23351521.

Overlack N, Goldmann T, Wolfrum U*, Nagel-Wolfrum K* (2012) Gene repair of an Usher syndrome causing mutation by zinc finger nucleases mediated homologous recombination. Invest Ophthalmol Visual Sci 53:4140-6. PMID: 22661463 (*equal contributions)

Jaillard C, Mouret A, Niepon ML, Clérin E, Yang Y, Lee-Rivera I, Aït-Ali N, Millet-Puel G, Cronin T, Sedmak T, Raffelsberger W, Kinzel B, Trembleau A, Poch O, Bennett J, Wolfrum U, Lledo PM, Sahel JA, Léveillard T (2012) Nxnl2 splicing results in dual functions in neuronal cell survival and maintenance of cell integrity. Hum Mol Genet 21:2298-311. PMID: 22343139.

Wolfrum U (2012) Usher-Syndrom Proteinnetzwerke. In: U. Horsch und A. Wanka (Hrsg.) Das Usher-Syndrome – eine erworbene Hörsehbehinterung. Ernst Reinhardt Verlag, München Basel, pp 57-69. (Review)

Jahn MR, Shukoor I, Tremel W, Wolfrum U, Kolb U, Nawroth T, Langguth P (2011) Hemin-coupled iron(III)-hydroxide nanoparticles show increased uptake in Caco-2 cells. J Pharm Pharmacol 63:1522-30. PMID: 22060282.

Sedmak T and Wolfrum U (2011) Intraflagellar transport protein in the ciliogenesis of photoreceptor cells. Biol Cell 103: 449-466. PMID: 21732910.

Overlack N, Kilic D, Bauß K, Maerker T, van Wijk E, Kremer H, Wolfrum U (2011) Direct interaction between Usher syndrome 1G protein SANS and myomegalin in mammalian photoreceptor cells. Biochimica et Biophysica Acta - Mol Cell Res 1813: 1883-1893. PMID: 21767579.

Gamerdinger M, Kaya AM, Wolfrum U, Clement AM, Behl C (2011) BAG3 mediates chaperone-based aggresome-targeting and selective autophagy of misfolded proteins. EMBO Rep 12:149-56. PMID: 21252941.

Goldmann T, Overlack N, Wolfrum U*, Nagel-Wolfrum K* (2011) PTC124-mediated translational read-through of a nonsense mutation causing Usher type 1C. Hum Gene Ther 22:537-47. PMID: 21235327 (*equal contributions)

Millington-Ward S, Chadderton N, O'Reilly M, Palfi A, Goldmann T, Kilty C, Humphries M, Wolfrum U, Bennett J, Humphries P, Kenna P, Farrar G (2011) Suppression and Replacement Gene Therapy for Autosomal Dominant Disease in a Murine Model of Dominant Retinitis Pigmentosa. Mol Ther 19:642-9. PMID: 21224835.

Chakarova CF, Khanna H, Shah AZ, Patil SB, Sedmak T, Murga-Zamalloa CA, Papaioannou MG, Nagel-Wolfrum K, Lopez I, Munro P, Cheetham M, Koenekoop RK, Rios RM, Matter K, Wolfrum U, Swaroop A, Bhattacharya SS (2011) TOPORS, implicated in retinal degeneration, is a cilia-centrosomal protein. Hum Mol Genet 20:975-87. PMID: 21159800.

Paquet-Durand F, Beck S, Michalakis S , Goldmann T, Huber G, Mühlfriedel R, Trifunovic D, Fischer MD, Fahl E, Duetsch G, Becirovic E, Wolfrum U, van Veen T, Biel M, Tanimoto N, Seeliger NW (2011) A key role for cyclic–nucleotide gated (CNG) channels in cGMP-related retinitis pigmentosa. Hum Mol Genet 20:941-7. PMID: 21149284.

Wolfrum U (2011) Protein networks related to the Usher syndrome gain insights in the molecular basis of the disease. In: Satpal Ahuja (ed) Usher Syndrome: Pathogenesis, Diagnosis and Therapy. Nova Science Publishers, Inc. USA, Chapter 4, pp 51-73. (Review)

Overlack N, Goldmann T, Wolfrum U, Nagel-Wolfrum K, (2011) Current therapeutic strategies for human Usher syndrome. In: Satpal Ahuja (ed) Usher Syndrome: Pathogenesis, Diagnosis and Therapy. Nova Science Publishers, Inc. USA., Chapter 22, pp 377-395. (Review)

Goldmann T, Rebibo-Sabbah A, Overlack N, Nudelman I, Belakhov V, Baasov T, Ben-Yosef T, Wolfrum U*, Nagel-Wolfrum K* (2010) Beneficial read-through of a USH1C nonsense mutation by designed aminoglycoside NB30 in the retina. Invest Ophthalmol Visual Sci 51:6671-80. PMID: 20671281 (*equal contributions)

Becke S, Aue S, Thomas D,Schader S, Podlech J, Bopp T, Sedmak T, Wolfrum U, Plachter B, Reyda S (2010) Optimized recombinant dense bodies of human cytomegalovirus efficiently prime virus specific lymphocytes and neutralizing antibodies without the addition of adjuvant. Vaccine 28:6191-8. PMID: 20655401.

Becke S, Fabre-Merssmann V, Aue S, Auerochs S, Sedmak T, Wolfrum U, Strand D, Marschall M, Plachter B, Reyda S (2010) Modification of the major tegument protein pp65 of human cytomegalovirus inhibits viral growth and leads to the enhancement of a protein complex with pUL69 and pUL97 in infected cells. J Gen Virol 91:2531-41. PMID: 20592110.

Yefimova MG, Messaddeq N, Karam A, Jacquard C, Weber C, Jonet L, Wolfrum U, Jeanny JC, Trottier Y (2010) Polyglutamine toxicity induces rod photoreceptor division, morphological transformation or death in Spinocerebellar ataxia 7 mouse retina. Neurobiol Dis 40:311-24. PMID: 20600911.

Sedmak T and Wolfrum U (2010) Intraflagellar transport molecules in ciliary and non-ciliary cells of the retina. J Cell Biol 189:171-186. PMID: 20368623.

Evans RJ, Schwarz N, Nagel-Wolfrum K, Wolfrum U, Hardcastle A, Cheetham ME (2010) The retinitis pigmentosa protein RP2 links pericentriolar vesicle transport between the Golgi and the primary cilium. Hum Mol Genet 19: 1358-1367. PMID: 20106869.

Orisme W, Li J, Goldmann T, Bolch S, Wolfrum U, Smith CW (2010) Light-dependent translocation of arrestin in rod photoreceptors is signaled through a phospholipase C cascade and requires ATP. Cell Signal 22:447-456. PMID: 19887106.

Jaissle GB, May CA, van de Pavert S, Wenzel A, Claes E, Reidel B, Szurman P, Haverkamp S, Fischer MD, Wolfrum U, Wijnholds J, Remé CE, Lütjen-Drecoll E, Humphries C, Seeliger MW (2010) Bone spicule formation in retinitis pigmentosa: Insights from a mouse model. Graefes Arch Clin Exp Ophthalmol 248:1063-70. PMID: 20012642.

Kersten FF, van Wijk E, van Reeuwijk J, van der Zwaag B, Märker T, Peters TA, Katsanis N, Wolfrum U, Keunen JEE, Roepman R, Kremer H (2010) Association of whirlin with Cav1.3 (alpha1D) channels in photoreceptors, defining a novel member of the usher protein network. Invest Ophthalmol Visual Sci 51: 2338-2346. PMID: 19959638.

Palfi A, Millington-Ward S, Chadderton NC, O’Reilly M, Goldmann T, Humphries MM, Wolfrum U, Humphries P, Kenna PF, Farrar GJ (2010) AAV-Mediated Rhodopsin Replacement Provides Therapeutic Benefit in Rhodopsin Knockout Mice. Hum Gene Ther 21: 311-323. PMID: 19824806.

Overlack N, Nagel-Wolfrum K, Wolfrum U (2010) The role of cadherins in sensory cell function In: Yoshida K (ed) Molecular and Functional Diversities of Cadherin and Protocadherin. Chapter 17, pp 259-272. (Review)

Lagziel A, Overlack N, Bernstein SL, Morell RJ, Wolfrum U, Friedman TB (2009) Expression of cadherin 23 isoforms is not conserved: implications for a mouse model of Usher syndrome type 1D. Mol Vis 15:1843-1857. PMID: 19756182.

Thissen MC, Krieglstein J, Wolfrum U, Klumpp S (2009) Dephosphorylation of Centrins by Protein Phosphatase 2C α and β. Res Lett Biochem. Article ID 685342. PMID: 22820751.

Insinna C, Humby M, Sedmak T, Wolfrum U, Besharse JC (2009) Different roles for KIF17 and kinesin II in photoreceptor development and maintenance. Dev Dyn 238:2211-22. PMID: 19384852.

Gamerdinger M, Hajieva P, Kaya AM, Wolfrum U, Hartl FU, Behl C (2009) Protein quality control during aging involves recruitment of the macroautophagy pathway by BAG3. EMBO J 28:889-901. PMID: 19229298.

Schneider E, Märker T, Daser A, Frey-Mahn G, Beyer V, Farcas R, Schneider-Rätzke B, Kohlschmidt N, Grossmann B, Bauss K, Napiontek U, Keilmann A, Bartsch O, Zechner U, Wolfrum U, Haaf T (2009) Homozygous disruption of PDZD7 by reciprocal translocation in a consanguineous family: a new member of the Usher syndrome protein interactome causing congenital hearing impairment. Hum Mol Genet 18:655-666. PMID: 19028668.

Specht D, Wu S-B, Turner P, Dearden P, Koentgen F, Wolfrum U, Maw M, Brandstätter JH, tom Dieck S (2009) Effects of presynaptic mutations on a postsynaptic Cacna1s calcium channel co-localized with mGluR6 at mouse photoreceptor ribbon synapses. Invest Ophthalmol Visual Sci 50:505-515. PMID:18952919.

van Wijk E, Kersten FF, Kartono A, Mans DA, Brandwijk K, Letteboer SJ, Peters TA, Märker T, Yan X, Cremers CW, Cremers F, Wolfrum U, Roepman R, Kremer H (2009) Usher syndrome and Leber congenital amaurosis are molecularly linked via a novel isoform of the centrosomal ninein-like protein. Hum Mol Genet 18:51-64. PMID: 18826961.

Sedmak T, Sehn E, Wolfrum U (2009) Immunoelectron microscopy of vesicle trafficking to the primary cilium of photoreceptor cells. In: Sloboda R (ed) Methods in Cell Biology: The Cell Biology of Primary Cilia. Elsevier Inc, Academic Press, Vol. 94, Chapter 13, pp 259-72. PMID: 20362095 (Review)

Reidel B, Goldmann T, Gießl A, Wolfrum U (2008) The translocation of signaling molecules in dark adapting mammalian rod photoreceptor cells is dependent on the cytoskeleton. Cell Motil Cytoskeleton 65:785-800. PMID: 18623243.

Feiden S, Wolfrum U, Wegener G, Kamp G (2008) Expression and compartmentalisation of the glycolytic enzymes GAPDH and pyruvate kinase in boar spermatogenesis. Reprod Fertil Dev 20:713-723. PMID: 18671919.

Spiwoks-Becker I, Maus C, tom Dieck S, Fejtová A, Engel L, Wolloscheck T, Wolfrum U, Vollrath L, Spessert R (2008) Active zone proteins are dynamically associated with synaptic ribbons in rat pinealocytes. Cell Tissue Res 333:185-195. PMID: 18523806.

Grube S, Wolfrum U, Langguth P (2008) Characterization of the epithelial permeation enhancing effect of basic butylated methacrylate copolymer - in vitro studies. Biomacromolecules 9:1398-1405. PMID: 18407686.

Lefèvre G, Michel V, Weil D, Lepelletier L, Bizard E, Wolfrum U, Hardelin J-P, Petit C (2008) A core cochlear phenotype in USH1 mouse mutants implicates fibrous links of the hair bundle in its cohesion, orientation, and differential growth. Development 135:1427-1437. PMID: 18339676.

Mersseman V, Böhm V, Holtappels R, Deegen P, Wolfrum U, Plachter B, Reyda S (2008) Refinement of strategies for the development of a human cytomegalovirus dense body vaccine. Med Microbiol Immunol 197: 97-107. PMID: 18320219.

Trojan P*, Rausch S*, Gießl A, Klemm C, Krause E, Pulvermüller A^§, Wolfrum U^§ (2008) Light-dependent CKII-mediated phosphorylation regulates centrin-G-protein complex formation with retinal visual G-protein. Biochimica et Biophysica Acta - Mol Cell Res 1783: 1248-1260. (both *first and ^§senior authors contributed equally to the work). PMID: 18269917. PMID: 18269917.

Mersseman V, Besold K, Reddehase MJ, Wolfrum U, Strand D, Plachter B, Reyda S (2008) Exogenous introduction of an immunodominant peptide from the non-structural IE1 protein of human cytomegalovirus into the MHC class I presentation pathway by recombinant dense bodies. J Gen Virol 89:369-379. PMID: 18198367.

Maerker T, van Wijk E, Overlack N, Kersten FFJ, McGee J, Goldmann T, Sehn E, Roepman R, Walsh EJ, Kremer H, Wolfrum U (2008) A novel Usher protein network at the periciliary reloading point between molecular transport machineries in vertebrate photoreceptor cells. Hum Mol Genet 17:71-86. PMID: 17906286.

Overlack N*, Märker T*, Latz, M, Nagel-Wolfrum, K, Wolfrum U (2008) SANS (USH1G) expression in developing and mature mammalian retina. Vision Res 48:400-412. PMID: 17923142 (*first authors contributed equally to the work)

Trojan P, Gießl A, Choe H-W, Krauß N, Pulvermüller A, Wolfrum U (2008). Centrins in retinal photoreceptor cells: regulators in the connecting cilium. Prog Retinal Eye Res 27: 237-259. PMID: 18329314 (Review)

Glube N, Gießl A, Wolfrum U, Langguth P (2007) Caki-1 cells represent an in vitro model system for studying the human proximal tubule epithelium. Nephron Exp Nephrol 107:e47-e56. PMID: 17804913.

Feiden S, Stypa H, Wolfrum U, Wegener G, Kamp G (2007) A novel pyruvate kinase (PK-S) from boar spermatozoa is localized at the fibrous sheath and the acrosome. Reproduction 134:81-95. PMID: 17641091.

Gosens I, Van Wijk E, Kersten F, Krieger E, Van der Zwaag B, Märker T, Letteboer SJF, Dusseljee S, Peters T, Spierenburg HA, Punte IM, Wolfrum U, Cremers FPM, Kremer H, Roepman R (2007) MPP1 links the Usher protein network and the Crumbs protein complex in the retina. Hum Mol Genet 16:1993-2003. PMID: 17584769.

Arts HH, Doherty D, van Beersum SEC, Parisi M, Letteboer SJF, Gorden NT, Peters TA, Märker T, Voesenek K, Kartono A, Ozyurek H, Farin FM, Kroes HY, Wolfrum U, Brunner HG, Cremers FPM, Glass IA, Knoers NVAM, Roepman R (2007) Mutations in the gene encoding the basal body protein KIAA1005, a novel nephrocystin-4 interactor, cause Joubert syndrome. Nat Genet 39:882-888. PMID: 17558407.

den Hollander AI., Koenekoo RK, Mohamed MD, Arts HH, Boldt K, Towns KV, Sedmak T, Beer M, Nagel-Wolfrum K, McKibbin M, Dharmaraj S, Lopez I, Ivings L, Williams GA, Springell K, Woods CG, Jafri H, Rashid Y, Strom TM, van der Zwaag B, Gosens I, Kersten FFJ, van Wijk E, Veltman JA, Zonneveld MN, van Beersum SEC, Maumenee IH, Wolfrum U, Cheetham ME, Ueffing M, Cremers FPM, Inglehearn CF, Roepman R (2007) Mutations in LCA5, encoding the ciliary protein lebercilin, cause Leber congenital amaurosis. Nat Genet 39:889–895. PMID: 17546029.

Hyenne V, Harf JC, Latz M, Maro B, Wolfrum U*, Simmler M-C* (2007) Vezatin, a ubiquitous protein of adherens cell-cell junctions, is exclusively expressed in germ cells in mouse testis. Reproduction 133:563-574. PMID: 17379651 (*both senior authors contributed equally to the work)

Roepman, R and Wolfrum U (2007) Protein networks and complexes in photoreceptor cilia.
In: Bertrand E and Faupel M (eds) Subcellular Proteomics Cell Deconstruction System Reconstruction Series: Subcellular Biochemistry, Vol. 43, Chapter 10, pp 209-235. PMID: 17953396 (Review)

Park JH, Pulvermüller A, Scheerer P, Rausch S, Gießl A, Höhne W, Wolfrum U, Hofmann K P, Ernst O P, Choe H-W, Krauß N (2006) Insights into functional aspects of centrins from the structure of N-terminally extended mouse centrin 1. Vision Res 46:4568-4574. PMID: 17027898.

Gießl A, Trojan P, Rausch S, Pulvermüller A, Wolfrum U (2006) Centrins, gatekeepers for the light-dependent translocation of transducin through the photoreceptor cell connecting cilium. Vision Res 46:4502-4509. PMID: 17027897.

Reidel B, Orisme W, Goldmann T, Smith Clay W, Wolfrum U (2006) Photoreceptor vitality in organotypic cultures of mature vertebrate retinas validated by light-dependent molecular movements. Vision Res 46:4464-4471. PMID: 16979692.

van Wijk E, van der Zwaag B, Peters T, Zimmerman U, te Brinke H, Kersten FFJ , Märker T, Aller E, Hoefsloot LH , Cremers CWRJ, Cremers FPM , Wolfrum U, Knipper M, Roepman R, Kremer H (2006) The DFNB31 gene product whirlin connects to the Usher protein network in cochlea and retina by direct association with USH2A and VLGR1. Hum Mol Genet 15:751-765. PMID: 16434480.

Reiners J and Wolfrum U (2006) Molecular analysis of the supramolecular Usher protein complex in the retina: harmonin as the key protein of the Usher syndrome. Adv Exp Med Biol 572:349-353. PMID: 17249595 (Review)

Kremer H, van Wijk E, T. Märker, Wolfrum U, Roepman R (2006) Usher syndrome: molecular links of pathogenesis, proteins and pathways. Hum Mol Genet 15 Suppl 2: R262-70. PMID: 16987892 (Review)

Reiners J*, Nagel-Wolfrum K*, Jürgens K, Märker T, Wolfrum U (2006) Molecular basis of human Usher syndrome: deciphering the meshes of the Usher protein network provides insights into the pathomechanisms of the Usher disease. Exp Eye Res 83:97-119
(*equal contributions). PMID: 16545802 (Review)

Reiners J*, van Wijk E*, Märker T, Zimmermann U, Jürgens K, te Brinke H, Overlack, N, Roepman R, Knipper M, Kremer H, Wolfrum U (2005) Scaffold protein harmonin (USH1C) provides molecular links between Usher syndrome type 1 and type 2. Hum Mol Genet 14:3933-3943. PMID: 16301216 (*both authors contributed equally to the work)

Bentmann A, Schmidt M, Reuss S, Wolfrum U, Hankeln T, Burmester T (2005) Divergent distribution in vascular and avascular mammalian retinae links neuroglobin to cellular respiration. J Biol Chem 280:20660-5. PMID: 15793311.

Reiners J, Märker T, Jürgens K, Reidel B, Wolfrum U (2005) Photoreceptor expression of the Usher syndrome type 1 protein protocadherin 15 (USH1F) and its interaction with the scaffold protein harmonin (USH1C). Mol Vis 11:347-355. PMID: 15928608.

Shu X, Fry, AM, Tulloch B, Manson FDC, Crabb JW, Khanna H, Faragher AJ, Lennon A, He S, Trojan P, Giessl A, Wolfrum U, Vervoort R, Swaroop A, Wright AF (2005) RPGR ORF15 isoform co-localizes with RPGRIP1 at centrioles and basal bodies and interacts with nucleophosmin. Hum Mol Genet 14:1183-1197. PMID: 15772089.

Michel V*, Goodyear RJ*, Weil D, Marcotti W, Perfettini I, Wolfrum U, Kros CJ, Richardson GP^#, Petit C^# (2005) Cadherin 23 is a component of the transient lateral links in the developing hair bundles of cochlear sensory cells. Dev Biol 280:281-294. PMID: 15882573 (*both authors contributed equally to the work; ^#shared senior authorship)

Adato A, Michel V, Kikkawa Y, Reiners J, Alagramam KN, Weil D, Yonekawa H, Wolfrum U, El-Amraoui A, Petit C (2005) Interactions in the network of Usher syndrome type 1 proteins. Hum Mol Genet 14:347-356. PMID: 15590703.

Park JH, Krauss N, Pulvermüller A, Scheerer P, Höhne W, Giessl A, Wolfrum U, Hofmann KP, Ernst OP, Choe H-W (2005) Crystallization and preliminary X-ray studies of mouse centrin1. Acta Cryst F61:510-513. PMID: 16511082.

Bytyqi AH, Lockridge O, Duysen E, Wang Y, Wolfrum U, Layer PG (2004) Impaired formation of the inner retina in an AChE knockout mouse results in degeneration of all photoreceptors. Europ J Neurosci 20:2953-2962. PMID: 15579149.

Giessl A, Pulvermüller A, Trojan P, Park JK, Choe H-W, Ernst OP, Hofmann KP, Wolfrum U (2004) Differential expression and interaction with the visual G-protein transducin of centrin isoforms in mammalian photoreceptor cells. J Biol Chem 279:51472-51481. PMID: 15347651.

Wolfrum U and Reiners J (2004) Myosin VIIa in photoreceptor cell synapses may contribute to an Usher 1 protein complex in the retina. IOVS eLetters (May 26 2004) http://www.iovs.org/cgi/eletters/44/11/5006.

Giessl A, Trojan P, Pulvermüller A, Wolfrum U (2004) Centrins, potential regulators of transducin translocation in photoreceptor cells. In: Williams DS (ed) Cell Biology and Related Disease of the Outer Retina. World Scientific Publishing Company Pte Ltd, Singapore. 195-222. (Review)

Reiners J, Reidel B, El-Amraoui A, Boëda B, Huber I, Petit C, Wolfrum U (2003) Differential distribution of harmonin isoforms and their possible role in Usher 1 protein complexes in mammalian photoreceptor cells. Invest Ophthalmol Visual Sci 44:5006-5015. PMID: 14578428.

Bode C and Wolfrum U (2003) Caspase-3 inhibitor reduces apoptotic photoreceptor cell death during inherited retinal degeneration in the tubby mouse. Mol Vis 9:144-150. PMID: 12724642.

Schmidt M*, Giessl A*, Laufs T, Hankeln T, Wolfrum U, Burmester T (2003) How does the eye breathe? Evidence for neuroglobin-mediated oxygen supply of the vertebrate retina. J Biol Chem 278:1932-1935. PMID: 12409290 (*both authors contributed equally to the work)

Wolfrum U (2003) The cellular function of the Usher gene product myosin VIIa is specified by its ligands. Adv Exp Med Biol 533:133-42. PMID: 15180257(Review)

Wolfrum U (2003) Die Rolle der Apoptose bei Netzhautdegenerationen. Medgen 15:124-128. (Review)

Boëda B, El-Amraoui A, Bahloul A, Goodyear R, Daviet L, Blanchard S, Perfettini I, Fath KR, Shorte S, Reiners J, Houdusse A, Legrain P, Wolfrum U, Richardson GP, Petit C (2002) Cadherin 23, harmonin, and myosin VIIa, three Usher I gene products that cooperate to shape the sensory hair cell bundle. EMBO J 21:6689-6699. PMID: 12485990.

Wolfrum U, Giessl A, Pulvermüller A (2002) Centrins in vertebrate photoreceptor cells. Adv Exp Med Biol 514:179-203. PMID: 12696921.

Bolz H, Reiners J, Wolfrum U, Gal A (2002) The role of cadherins in Ca²⁺-mediated cell adhesion and inherited photoreceptor degeneration. Adv Exp Med Biol 514:399-410. PMID: 12596935.

El-Amraoui A, Schonn J-S, Küssel-Andermann P, Blanchard S, Desnos C, Henry J-P, Wolfrum U, Darchen F, Petit C (2002) MyRIP, a novel Rab effector, enables myosin VIIA recruitement to melanosomes. EMBO Reports 3:463-470. PMID: 11964381.

Pulvermüller A, Giessl A, Heck M, Wottrich R, Schmitt A, Ernst OP, Choe H-W, Hofmann KP, Wolfrum U (2002) Calcium dependent assembly of centrin/G-protein complex in photoreceptor cells. Mol Cell Biol 22:2194-2203. PMID: 11884606.

Schmitt A and Wolfrum U (2001) Identification of novel molecular components of the photoreceptor connecting cilium by immunoscreens. Exp Eye Res 73:837-849. PMID: 11846514.

Küssel-Andermann P, El-Amraoui A, Safieddine S, Nouaille S, Perfettini I, Lecuit M, Cossart P, Wolfrum U, Petit C (2000) Vezatin, a novel transmembrane protein, bridges myosin VIIA to the cadherin/catenin complex. EMBO J 19:6020-6029. PMID: 11080149.

Wolfrum U and Schmitt A (2000) Rhodopsin transport in the membrane of the connecting cilium of mammalian photoreceptor cells. Cell Motil Cytoskeleton 46:95-107. PMID: 10891855.

Remé CE*, Wolfrum U*, Imsand C, Hafezi F, Williams TP (1999) Photoreceptor autophagy: Effects of light-history on number and opsin content of degradative vacuoles. Invest Ophthalmol Visual Sci 40:2398-2404. PMID: 10476808 (*both authors contributed equally to the work).

Körschen HG, Beyermann M, Müller F, Heck M, Vantler M, Koch KW, Kellner R, Wolfrum U, Bode C, Hofmann KP, Kaupp UB (1999) Interaction of glutamic acid-rich proteins with components of the cGMP-signaling pathway in rod photoreceptors. Nature 400:761-766. PMID: 10466724.

Tai AW, Chuang J-Z, Bode C, Wolfrum U, Sung C-H (1999) Rhodopsin´s carboxy-terminal cytoplasmic tail acts as a membrane receptor for cytoplasmic dynein by binding the dynein light chain Tctex-1. Cell 97:877-887. PMID: 10399916.

Wolfrum U and Schmitt A (1999) Evidence for myosin VIIa driven transport of rhodopsin in the plasma membrane of the photoreceptor-connecting cilium. In: Hollyfield JG, Andersson RE, LaVail M (eds) Retinal degeneration diseases and experimental therapy. Kluwer Academic / Plenum Publishers, New York. 3-14. (Review)

Wolfrum U, Liu XR, Schmitt A, Udovichenko IP, Williams DS (1998) Myosin VIIa as a common component of cilia and microvilli. Cell Motil Cytoskeleton 40:261-271. PMID: 9678669.

Wolfrum U and Salisbury JL (1998) Expression of centrin isoforms in the mammalian retina. Exp Cell Res 242:10-17. PMID: 9665797.

Liu X, Vansant G, Udovichenko I P, Wolfrum U, Williams DS (1997) Myosin VIIa, the product of the Usher 1B syndrome gene, is concentrated in the connecting cilia of rod and cone photoreceptor cells. Cell Motil Cytoskeleton 37:240-252. PMID: 9227854.

Wolfrum U (1997) Cryofixation methods in biological electron microscopy - their limits. J Comp Assis Microscopy 9:73-76.

Huber A, Schulz S, Bentrop J, Groell C, Wolfrum U, Paulsen R (1997) Molecular cloning of Drosophila Rh6 rhodopsin: the visual pigment of a subset of R8 photoreceptor cells. FEBS Letters 406:6-10. PMID: 9109375.

Wolfrum U (1997) Cytoskeletal elements in insect sensilla. J Insect Morphol & Embryol 26:191-203. PMID: (Review)

Huber A, Sander P, Wolfrum U, Groell C, Gerdon G, Paulsen R (1996) Isolation of genes encoding photoreceptor-specific proteins by immunoscreening with antibodies against purified blowfly rhabdomes. J Photochem Photobiol 35:69-76. PMID: 8823935.

Wolfrum U (1995) Centrin in the photoreceptor cells of mammalian retinae. Cell Motil Cytoskeleton 32:55-64. PMID: 8674134.

Huber A, Wolfrum U, Paulsen, R (1994) Opsin maturation and targeting to rhabdomeral photoreceptor membranes requires the retinal chromophore. Eur J Cell Biol 63:219-229. PMID: 8082646.

Kaib M, Ziesmann J, Wolfrum U (1993) Modulation of odour-sensitivity by CO2 in a termite sensillum: A possible mechanism for signal interpretation. In: Wiese K, Gribakin FG, Popov AV, Renninger G (eds) Sensory systems of arthropods. Brinkhäuser, Basel. 481-488.

Wolfrum U (1992) Cytoskeletal elements in arthropod sensilla and mammalian photoreceptors. Biol Cell 76:373-381. PMID: 1305480.

Wolfrum U (1991) Distribution of F-actin in the compound eye of Calliphora erythrocephala (Diptera, Insecta). Cell Tissue Res 263:399-403.

Wolfrum U (1991) Tropomyosin is co-localized with the actin filaments of the scolopale in insect sensilla. Cell Tissue Res 265:11-17.

Wolfrum U (1991) Centrin- and a-actinin-like immunoreactivity in the ciliary rootlets of insect sensilla. Cell Tissue Res 266:31-38.

Wolfrum U (1990) Actin filaments: the main components of the scolopale in insect sensilla. Cell Tissue Res 261:85-96.

Wolfrum U and Kaib M (1988) Kastenspezifisches Verhalten der Termite Schedorhinotermes lamanianus und dessen Beziehung zu Unterschieden in Ultrastruktur, Häufigkeit und Topographie antennaler Sensillen. Mitt dtsch Ges allg angew Ent 6:86-90.