LDB3 splicing abnormalities are specific to skeletal muscles of patients with myotonic dystrophy type 1 and alter its PKC binding affinity

LDB3 splicing abnormalities are specific to skeletal muscles of patients with myotonic dystrophy type 1 and alter its PKC binding affinity

Myotonic dystrophy type 1 (DM1) is caused by transcription of CUG repeat RNA, which causes sequestration of muscleblind-like 1 (MBNL1) and upregulation of CUG triplet repeat RNA-binding protein (CUG-BP1). In DM1, dysregulation of these proteins contributes to many aberrant splicing events, causing v...

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Bibliographic Details
Main Authors: Yoshihiro Yamashita, Tohru Matsuura, Tatsuaki Kurosaki, Yoshinobu Amakusa, Masanobu Kinoshita, Tohru Ibi, Ko Sahashi, Kinji Ohno
Format: Article
Language:English
Published: Elsevier 2014-09-01
Series:Neurobiology of Disease
Subjects:
Myotonic dystrophy type 1
Muscleblind-like 1
CUG triplet repeat RNA-binding protein
LIM domain binding 3
Protein kinase C
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996114001442

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http://www.sciencedirect.com/science/article/pii/S0969996114001442

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