Muscleblind-Like 1 and Muscleblind-Like 3 Depletion Synergistically Enhances Myotonia by Altering Clc-1 RNA Translation

Muscleblind-Like 1 and Muscleblind-Like 3 Depletion Synergistically Enhances Myotonia by Altering Clc-1 RNA Translation

Loss of Muscleblind-like 1 (Mbnl1) is known to alter Clc-1 splicing to result in myotonia. Mbnl1ΔE3/ΔE3/Mbnl3ΔE2 mice, depleted of Mbnl1 and Mbnl3, demonstrate a profound enhancement of myotonia and an increase in the number of muscle fibers with very low Clc-1 currents, where gClmax values approach...

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Bibliographic Details
Main Authors: Jongkyu Choi, Kirkwood E. Personius, Marino DiFranco, Warunee Dansithong, Carl Yu, Saumya Srivastava, Donald M. Dixon, Darshan B. Bhatt, Lucio Comai, Julio L. Vergara, Sita Reddy
Format: Article
Language:English
Published: Elsevier 2015-09-01
Series:EBioMedicine
Subjects:
Muscleblind-like 1
Muscleblind-like 2
Myotonia
Clc-1
RNA translation
Online Access:http://www.sciencedirect.com/science/article/pii/S2352396415300803

Internet

http://www.sciencedirect.com/science/article/pii/S2352396415300803

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