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Rpgrip1 is required for rod outer segment development and ciliary protein trafficking in zebrafish

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Author(s)

Rakesh K. Raghupathy, Xun Zhang, Fei Liu, Reem H. Alhasani, Lincoln Biswas, Saeed Akhtar, Luyuan Pan, Cecilia B. Moens, Wenchang Li, Mugen Liu, Breandan N. Kennedy, Xinhua Shu

School/Research organisations

Abstract

Mutations in the RPGR-interacting protein 1 (RPGRIP1) gene cause recessive Leber congenital amaurosis (LCA), juvenile retinitis pigmentosa (RP) and cone-rod dystrophy. RPGRIP1 interacts with other retinal disease-causing proteins and has been proposed to have a role in ciliary protein transport; however, its function remains elusive. Here, we describe a new zebrafish model carrying a nonsense mutation in the rpgrip1 gene. Rpgrip1homozygous mutants do not form rod outer segments and display mislocalization of rhodopsin, suggesting a role for RPGRIP1 in rhodopsin-bearing vesicle trafficking. Furthermore, Rab8, the key regulator of rhodopsin ciliary trafficking, was mislocalized in photoreceptor cells of rpgrip1 mutants. The degeneration of rod cells is early onset, followed by the death of cone cells. These phenotypes are similar to that observed in LCA and juvenile RP patients. Our data indicate RPGRIP1 is necessary for rod outer segment development through regulating ciliary protein trafficking. The rpgrip1 mutant zebrafish may provide a platform for developing therapeutic treatments for RP patients.

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Details

Original languageEnglish
Article number16881
Number of pages14
JournalScientific Reports
Volume7
Early online date4 Dec 2017
DOIs
Publication statusE-pub ahead of print - 4 Dec 2017

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