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The tectonic complex regulates membrane protein composition in the photoreceptor cilium

Truong HM, Cruz-Colón KO, Martínez-Márquez JY, Willer JR, Travis AM, Biswas SK, Lo WK, Bolz HJ, Pearring JN

Nature Communications. 2023; 14(1):5671. PMID:37704658

Nrl:CreERT2 mouse model to induce mosaic expression in rod photoreceptors

Thorson MT, Wei SE, Johnson C, Gabriel CJ, Arshavsky VY, Pearring JN

Frontiers in Molecular Neuroscience. 2023; 16:1161127. PMID: 37181654

CEP162 deficiency causes human retinal degeneration and reveals a dual role in ciliogenesis and neurogenesis

Nuzhat N, Van SchilK, Liakopoulos S, Bauwens M, Rey AD, Käseberg S, Jäger M, Willer JR, Winter J, Truong HM, Gruartmoner N, Van Heetvelde M, Wolf J, Merget R, Grasshoff-Derr S, Van Dorpe J, Hoorens A, Stöhr H, Mansard L, Roux AF, Langmann T, Dannhausen K, Rosenkranz D, Wissing KM, Van Lint M, Rossmann H, Häuser F, Nürnberg P,  Thiele H, Zechner U, Pearring JN*, De Baere E*, Bolz HJ*

Journal of Clinical Investigation. 2023; 133(8):e161156. PMID: 36862503

Mechanistic Analysis of CCP1 in Generating ΔC2 α-Tubulin in Mammalian Cells and Photoreceptor Neurons

Hotta T, PlemmonsA, Gebbie M, Ziehm TA, Blasius TL, Johnson C, Verhey KJ, Pearring JN, Ohi R

Biomolecules. 2023; 13(2):357.  PMID: 36830726

Disrupting the ciliary gradient of active Arl3 affects rod photoreceptor nuclear migration

Travis AM, Manocha S, Willer JR, Wessler TS, Skiba NP, Pearring JN

eLife. 2023; 12:e80533. PMID: 36598133

The GARP Domain of the rod CNG channel's β1-subunit contains distinct sites for outer segment targeting and connecting to the photoreceptor disk rim

Pearring JN, Martínez-Márquez J, Willer JR, Lieu EC, Salinas RY, Arshavsky VY.

Journal of Neuroscience. 2021; 41(14):3094-3104. PMID:33637563

Cover Image

This image shows photoreceptor neurons in a mouse retina. A subset of rod photoreceptors are labeled with mCherry (red) and are expressing the alpha (magenta) and beta (green) subunits of the rod cyclic nucleotide-gated (CNG) channel. In the article by Pearring et al, the authors identify distinct sites encoded within the beta subunit that are used to specifically target the CNG channel to the light-sensitive outer segment compartment and subsequently sequester the CNG channel into the plasma membrane surrounding the membrane discs.

Photoreceptor discs: built like ectosomes

Spencer WJ, Lewis TR, Pearring JN, Arshavsky VY.

Trends in Cell Biology. 2020; S0962-8924(20)30165-3. PMID: 32900570

PRCD is essential for high-fidelity photoreceptor disc formation.

Spencer WJ, Ding JD,Lewis TR, Yu C, Phan S, Pearring JN, Kim KY, Thor A, Mathew R, Kalnitsky J, Hao Y,Travis AM, Biswas SK, Lo WK, Besharse JC, Ellisman MH, Saban DR, Burns ME,Arshavsky VY.

PNAS. 2019; 116(26):13087-13096. PMID: 31189593

Cover Image

Pictured is an electron micrograph showing accumulation of extracellular vesicles around photoreceptor cells in a mouse lacking the Prcd gene. Mutations in Prcd are a common cause of blindness in dogs and linked to retinitis pigmentosa in humans. William J. Spencer et al. found that PRCD is necessary for light-sensitive membranes of photoreceptor cells to align, flatten, and assume the shape required for efficient light capture. In the absence of PRCD, the membranes bulge and release massive amounts of extracellular vesicles, causing inflammation and retinal pathology. 

Photoreceptor discs form through peripherin-dependent suppression of ciliary ectosome release

Salinas RY*, Pearring JN*, Ding JD*, Spencer WJ, Hao Y, Arshavsky VY.

Journal of Cell Biology. 2017; 216(5):1489-1499. PMID: 28381413

Loss of Arf4 causes severe degeneration of the exocrine pancreas but not cystic kidney disease or retinal degeneration

Pearring JN, San Agustin JT, Lobanova ES, Gabriel CJ, Lieu EC, Monis WJ, Stuck MW, Strittmatter L, Jaber SM, Arshavsky VY, Pazour GJ.

PLoS Genetics. 2017; 13(4):e1006740. PMID: 28410364

Dimerization deficiency of enigmatic retinitis pigmentosa-linked rhodopsin mutants

Ploier B, Caro LN, Morizumi T, Pandey K, Pearring JN, Goren MA, Finnemann SC, Graumann J, Arshavsky VY, Dittman JS, Ernst OP, Menon AK.

Nature Communications. 2016; 7:12832. PMID: 27694816

Progressive rod-cone degeneration (PRCD) protein requires N-terminal S-acylation and rhodopsin binding for photoreceptor outer segment localization and maintaining intracellular stability

Spencer WJ, Pearring JN, Salinas RY, Loiselle DR, Skiba NP, Arshavsky VY.

Biochemistry. 2016; 55(36):5028-37. PMID: 27509380

De novo GMNN mutations cause autosomal-dominant primordial dwarfism associated with Meier-Gorlin syndrome

Burrage LC, Charng WL, Eldomery MK, Willer JR, Davis EE, Lugtenberg D, Zhu W, Leduc MS, Akdemir ZC, Azamian M, Zapata G, Hernandez PP, Schoots J, de Munnik SA, Roepman R, Pearring JN, Jhangiani S, Katsanis N, Vissers LE, Brunner HG, Beaudet AL, Rosenfeld JA, Muzny DM, Gibbs RA, Eng CM, Xia F, Lalani SR, Lupski JR, Bongers EM, Yang Y.

American Journal of Human Genetics. 2015; 97(6):904-13. PMID: 26637980

Guanylate cyclase 1 relies on rhodopsin for intracellular stability and ciliary trafficking

Pearring JN, Spencer WJ, Lieu EC, Arshavsky VY. 

eLife. 2015; e12058. PMID: 26590321

R9AP targeting to rod outer segments is independent of rhodopsin and is guided by the SNARE homology domain

Pearring JN, Lieu EC, Winter JR, Baker SA, Arshavsky VY.

Molecular Biology of the Cell. 2014; 25(17):2644-9. PMID: 25009288

Protein sorting, targeting and trafficking in photoreceptor cells

Pearring JN, Salinas RY, Baker SA, Arshavsky VY.

Progress in Retinal and Eye Research. 2013; 36:24-51. PMID:23562855

Depolarizing bipolar cell dysfunction due to a Trpm1 point mutation

Peachey NS, Pearring JN, Bojang P Jr, Hirschtritt ME, Sturgill-Short G, Ray TA, Furukawa T, Koike C, Goldberg AF, Shen Y, McCall MA, Nawy S, Nishina PM, Gregg RG.

Journal of Neurophysiology. 2012; 108(9):2442-51. PMID: 22896717

GPR179 is required for depolarizing bipolar cell function and is mutated in autosomal-recessive complete congenital stationary night blindness

Peachey NS, Ray TA, Florijn R, Rowe LB, Sjoerdsma T, Contreras-Alcantara S, Baba K, Tosini G, Pozdeyev N, Iuvone PM, Bojang P Jr, Pearring JN, Simonsz HJ, van Genderen M, Birch DG, Traboulsi EI, Dorfman A, Lopez I, Ren H, Goldberg AF, Nishina PM, Lachapelle P, McCall MA, Koenekoop RK, Bergen AA, Kamermans M, Gregg RG.

American Journal of Human Genetics. 2012; 90(2):331-9. PMID: 22325362

A role for nyctalopin, a small leucine-rich repeat protein, in localizing the TRP melastatin 1 channel to retinal depolarizing bipolar cell dendrites

Pearring JN, Bojang P Jr, Shen Y, Koike C, Furukawa T, Nawy S, Gregg RG.

Journal of Neuroscience. 2011; 31(27):10060-6. PMID:21734298

Mutations in TRPM1 are a common cause of complete congenital stationary night blindness

van Genderen MM, Bijveld MM, Claassen YB, Florijn RJ, Pearring JN, Meire FM, McCall MA, Riemslag FC, Gregg RG, Bergen AA, Kamermans M.

American Journal of Human Genetics. 2009; 85(5):730-6. PMID:19896109

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