Nuclear RNA Foci in the Heart in Myotonic Dystrophy-Reference-Cited by-同舟云学术

Nuclear RNA Foci in the Heart in Myotonic Dystrophy

Published:2005-11-25 Issue:11 Volume:97 Page:1152-1155
ISSN:0009-7330
Container-title:Circulation Research
language:en
Short-container-title:Circulation Research

Author:

Mankodi Ami¹,Lin Xiaoyan¹,Blaxall Burns C.¹,Swanson Maurice S.¹,Thornton Charles A.¹

Affiliation:

1. From the Departments of Neurology (A.M., C.A.T.), Neuroscience (X.L.), and Cardiovascular Research Institute (B.C.B.), University of Rochester Medical Center, New York; and the Department of Molecular Genetics and Microbiology (M.S.S.), University of Florida, Gainesville.

Abstract

The disease mechanism underlying myotonic dystrophy type 1 (DM1) pathogenesis in skeletal muscle may involve sequestration of RNA binding proteins in nuclear foci of expanded poly(CUG) RNA. Here we report evidence for a parallel mechanism in the heart. Accumulation of expanded poly(CUG) RNA in nuclear foci is associated with sequestration of muscleblind proteins and abnormal regulation of alternative splicing in DM1 cardiac muscle. A toxic effect of RNA with an expanded repeat may contribute to cardiac disease in DM1.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Cardiology and Cardiovascular Medicine,Physiology

Link

https://www.ahajournals.org/doi/pdf/10.1161/01.RES.0000193598.89753.e3

Reference31 articles.

1. Cardiac abnormalities in myotonic dystrophy

2. Pathology of the cardiac conduction system in myotonic dystrophy: A study of 12 cases

3. MYOTONIC DYSTROPHY AND THE HEART

4. Age and causes of death in adult-onset myotonic dystrophy

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