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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
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
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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