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1.
Int J Mol Sci ; 25(8)2024 Apr 12.
Artículo en Inglés | MEDLINE | ID: mdl-38673863

RESUMEN

In this review, we outline our current understanding of the mechanisms involved in the absorption, storage, and transport of dietary vitamin A to the eye, and the trafficking of rhodopsin protein to the photoreceptor outer segments, which encompasses the logistical backbone required for photoreceptor cell function. Two key mechanisms of this process are emphasized in this manuscript: ocular and systemic vitamin A membrane transporters, and rhodopsin transporters. Understanding the complementary mechanisms responsible for the generation and proper transport of the retinylidene protein to the photoreceptor outer segment will eventually shed light on the importance of genes encoded by these proteins, and their relationship on normal visual function and in the pathophysiology of retinal degenerative diseases.


Asunto(s)
Rodopsina , Vitamina A , Rodopsina/metabolismo , Rodopsina/genética , Humanos , Vitamina A/metabolismo , Animales , Células Fotorreceptoras de Vertebrados/metabolismo , Células Fotorreceptoras/metabolismo , Transporte Biológico
2.
Adv Exp Med Biol ; 1415: 499-505, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37440078

RESUMEN

Rods and cones are photoreceptor neurons in the retina that are required for visual sensation in vertebrates, where proper protein localization and compartmentalization are critical for phototransduction and visual function. In human retinal diseases, improper protein transport to the outer segment (OS) or mislocalization of proteins to the inner segment (IS) could lead to impaired visual responses and photoreceptor cell degeneration, causing a loss of visual function. We showed involvement of an unconventional motor protein, MYO1C, in the proper localization of rhodopsin to the OS, where loss of MYO1C in a mammalian model caused mislocalization of rhodopsin to IS and cell bodies, leading to progressively severe retinal phenotypes. In this study, using modeling and docking analysis, we aimed to identify the protein-protein interaction sites between MYO1C and Rhodopsin to establish a hypothesis that a physical interaction between these proteins is necessary for the proper trafficking of rhodopsin and visual function.


Asunto(s)
Retina , Rodopsina , Animales , Humanos , Rodopsina/genética , Rodopsina/metabolismo , Retina/metabolismo , Células Fotorreceptoras Retinianas Bastones/metabolismo , Células Fotorreceptoras Retinianas Conos/metabolismo , Transporte de Proteínas/fisiología , Mamíferos/metabolismo , Miosina Tipo I/metabolismo
3.
Ophthalmic Genet ; 43(3): 285-300, 2022 06.
Artículo en Inglés | MEDLINE | ID: mdl-35470760

RESUMEN

BACKGROUND: Rods and cones are photoreceptor neurons in the retina that are required for visual sensation in vertebrates, wherein the perception of vision is initiated when these neurons respond to photons in the light stimuli. The photoreceptor cell is structurally studied as outer segments (OS) and inner segments (IS) where proper protein sorting, localization, and compartmentalization are critical for phototransduction, visual function, and survival. In human retinal diseases, improper protein transport to the OS or mislocalization of proteins to the IS and other cellular compartments could lead to impaired visual responses and photoreceptor cell degeneration that ultimately cause loss of visual function. RESULTS: Therefore, studying and identifying mechanisms involved in facilitating and maintaining proper protein transport in photoreceptor cells would help our understanding of pathologies involving retinal cell degeneration in inherited retinal dystrophies, age-related macular degeneration, and Usher Syndrome. CONCLUSIONS: Our mini-review will discuss mechanisms of protein transport within photoreceptors and introduce a novel role for an unconventional motor protein, MYO1C, in actin-based motor transport of the visual chromophore Rhodopsin to the OS, in support of phototransduction and visual function.


Asunto(s)
Degeneración Retiniana , Visión Ocular , Animales , Humanos , Transporte de Proteínas/fisiología , Retina , Células Fotorreceptoras Retinianas Conos/metabolismo , Células Fotorreceptoras Retinianas Bastones/metabolismo
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