NDLI: Impaired association of retinal degeneration-3 with guanylate cyclase-1 and guanylate cyclase-activating protein-1 leads to leber congenital amaurosis-1.

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Impaired association of retinal degeneration-3 with guanylate cyclase-1 and guanylate cyclase-activating protein-1 leads to leber congenital amaurosis-1.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Rajala, Raju V. S. Zulliger, Rahel Molday, Robert S. Azadi, Seifollah Naash, Muna I.
Description	Author Affiliation: Zulliger R ( From the Departments of Cell Biology.); Naash MI ( From the Departments of Cell Biology.); Rajala RV ( From the Departments of Cell Biology, Ophthalmology, and Physiology, University of Oklahoma Health Sciences Center and the Dean McGee Eye Institute, Oklahoma City, Oklahoma 73104 and.); Molday RS ( the Department of Biochemistry and Molecular Biology, University of British Columbia, Vancouver, British Columbia V6T 1Z3, Canada.); Azadi S ( From the Departments of Cell Biology, the Dean McGee Eye Institute, Oklahoma City, Oklahoma 73104 and sazadi@ouhsc.edu.)
Abstract	One-fifth of all cases of Leber congenital amaurosis are type 1 (LCA1). LCA1 is a severe form of retinal dystrophy caused by loss-of-function mutations in guanylate cyclase 1 (GC1), a key member of the phototransduction cascade involved in modulating the photocurrents. Although GC1 has been studied for some time, the mechanisms responsible for its regulation and membrane targeting are not fully understood. We reported earlier that retinal degeneration 3 (RD3) protein interacts with GC1 and promotes its targeting to the photoreceptor outer segments (POS). Here, we extend our studies to show a direct association between RD3 and guanylate cyclase activating protein 1 (GCAP1). Furthermore, we demonstrate that this functional interaction is important for GC1 targeting to POS. We also show that most LCA1-causing mutations in GC1 result in lost GC1 interaction with RD3 or GC1 being targeted to the plasma membrane. Our data suggest that GC1, GCAP1, and RD3 form a complex in the endoplasmic reticulum that targets GC1 to POS. Interruption of this assembly is likely the underlying mechanism for a subset of LCA1. This study offers insights for the development of therapeutic strategies to treat this severe form of blindness.
ISSN	00219258
e-ISSN	1083351X
Journal	Journal of Biological Chemistry
Issue Number	6
Volume Number	290
Language	English
Publisher	American Society for Biochemistry and Molecular Biology (United States)
Publisher Date	2015-02-06
Publisher Place	United States
Access Restriction	Open
Subject Keyword	Guanylate Cyclase-Activating Proteins Metabolism Guanylate Cyclase Leber Congenital Amaurosis Nuclear Proteins Receptors, Cell Surface Animals COS Cells Cell Membrane Cercopithecus Aethiops Endoplasmic Reticulum Eye Proteins Genetics Mice Protein Binding Protein Transport Retinal Photoreceptor Cell Outer Segment Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Biochemistry Molecular Biology
Content Type	Text
Resource Type	Article
Subject	Cell Biology Biochemistry Molecular Biology

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