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Comprehensive identification of mRNA isoforms reveals the diversity of neural cell-surface molecules with roles in retinal development and disease

Genes encoding cell-surface proteins control nervous system development and are implicated in neurological disorders. These genes produce alternative mRNA isoforms which remain poorly characterized, impeding understanding of how disease-associated mutations cause pathology. Here we introduce a strat...

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Detalles Bibliográficos
Autores principales: Ray, Thomas A., Cochran, Kelly, Kozlowski, Chris, Wang, Jingjing, Alexander, Graham, Cady, Martha A., Spencer, William J., Ruzycki, Philip A., Clark, Brian S., Laeremans, Annelies, He, Ming-Xiao, Wang, Xiaoming, Park, Emily, Hao, Ying, Iannaccone, Alessandro, Hu, Gary, Fedrigo, Olivier, Skiba, Nikolai P., Arshavsky, Vadim Y., Kay, Jeremy N.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7335077/
https://www.ncbi.nlm.nih.gov/pubmed/32620864
http://dx.doi.org/10.1038/s41467-020-17009-7
Descripción
Sumario:Genes encoding cell-surface proteins control nervous system development and are implicated in neurological disorders. These genes produce alternative mRNA isoforms which remain poorly characterized, impeding understanding of how disease-associated mutations cause pathology. Here we introduce a strategy to define complete portfolios of full-length isoforms encoded by individual genes. Applying this approach to neural cell-surface molecules, we identify thousands of unannotated isoforms expressed in retina and brain. By mass spectrometry we confirm expression of newly-discovered proteins on the cell surface in vivo. Remarkably, we discover that the major isoform of a retinal degeneration gene, CRB1, was previously overlooked. This CRB1 isoform is the only one expressed by photoreceptors, the affected cells in CRB1 disease. Using mouse mutants, we identify a function for this isoform at photoreceptor-glial junctions and demonstrate that loss of this isoform accelerates photoreceptor death. Therefore, our isoform identification strategy enables discovery of new gene functions relevant to disease.