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Syntrophin, alpha 1

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Title: Syntrophin, alpha 1  
Author: World Heritage Encyclopedia
Language: English
Subject: Dystrophin, Nav1.5, List of A1 genes, proteins or receptors
Publisher: World Heritage Encyclopedia

Syntrophin, alpha 1

Syntrophin, alpha 1
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Available structures
PDB Ortholog search: RCSB
SNTA1 Gene
RNA expression pattern

Alpha-1-syntrophin is a protein that in humans is encoded by the SNTA1 gene.[1][2][3]

Dystrophin is a large, rod-like cytoskeletal protein found at the inner surface of muscle fibers. Dystrophin is missing in Duchenne Muscular Dystrophy patients and is present in reduced amounts in Becker Muscular Dystrophy patients. The protein encoded by this gene is a peripheral membrane protein found associated with dystrophin and dystrophin-related proteins. This gene is a member of the syntrophin gene family, which contains at least two other structurally related genes.[3] The PDZ domain of syntrophin-α1(SNTA1), the most abundant isoform in the heart, has been reported to bind to the C-terminal domain of murine cardiac voltage-gated sodium channels (SkM2) causing altering ion channel activity leading to Long QT syndrome.[4][5]


Syntrophin, alpha 1 has been shown to interact with Dystrophin,[1][6][7] Nav1.1[7] and Nav1.5.[7]


Further reading

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