Expressing a Z-disk nebulin fragment in nebulin-deficient mouse muscle: effects on muscle structure and function

Expressing a Z-disk nebulin fragment in nebulin-deficient mouse muscle: effects on muscle structure and function

Abstract Background Nebulin is a critical thin filament-binding protein that spans from the Z-disk of the skeletal muscle sarcomere to near the pointed end of the thin filament. Its massive size and actin-binding property allows it to provide the thin filaments with structural and regulatory support...

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Bibliographic Details
Main Authors: Frank Li, Justin Kolb, Julie Crudele, Paola Tonino, Zaynab Hourani, John E. Smith, Jeffrey S. Chamberlain, Henk Granzier
Format: Article
Language:English
Published: BMC 2020-01-01
Series:Skeletal Muscle
Subjects:
Nebulin
Nemaline myopathy
Gene therapy
AAV
Sarcomere
Thin filament
Online Access:https://doi.org/10.1186/s13395-019-0219-9

Internet

https://doi.org/10.1186/s13395-019-0219-9

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