Reducing sarcolipin expression mitigates Duchenne muscular dystrophy and associated cardiomyopathy in mice

Antanina Voit; Vishwendra Patel; Ronald Pachon; Vikas Shah; Mohammad Bakhutma; Erik Kohlbrenner; Joseph J. McArdle; Louis J. Dell’Italia; Jerry R. Mendell; Lai-Hua Xie; Roger J. Hajjar; Dongsheng Duan; Diego Fraidenraich; Gopal J. Babu

doi:10.1038/s41467-017-01146-7

Nature Communications (Oct 2017)

Reducing sarcolipin expression mitigates Duchenne muscular dystrophy and associated cardiomyopathy in mice

Antanina Voit,
Vishwendra Patel,
Ronald Pachon,
Vikas Shah,
Mohammad Bakhutma,
Erik Kohlbrenner,
Joseph J. McArdle,
Louis J. Dell’Italia,
Jerry R. Mendell,
Lai-Hua Xie,
Roger J. Hajjar,
Dongsheng Duan,
Diego Fraidenraich,
Gopal J. Babu

Affiliations

Antanina Voit: Department of Cell Biology and Molecular Medicine, New Jersey Medical School, Rutgers, The State University of New Jersey
Vishwendra Patel: Department of Pharmacology, Physiology and Neuroscience, New Jersey Medical School, Rutgers, The State University of New Jersey
Ronald Pachon: Department of Cell Biology and Molecular Medicine, New Jersey Medical School, Rutgers, The State University of New Jersey
Vikas Shah: Department of Cell Biology and Molecular Medicine, New Jersey Medical School, Rutgers, The State University of New Jersey
Mohammad Bakhutma: Department of Cell Biology and Molecular Medicine, New Jersey Medical School, Rutgers, The State University of New Jersey
Erik Kohlbrenner: Cardiovascular Research Center, Icahn School of Medicine at Mount Sinai
Joseph J. McArdle: Department of Pharmacology, Physiology and Neuroscience, New Jersey Medical School, Rutgers, The State University of New Jersey
Louis J. Dell’Italia: Department of Medicine, University of Alabama at Birmingham, and Birmingham VA Medical Center
Jerry R. Mendell: Department of Pediatrics and Department of Neurology, Ohio State University Research Institute at Nationwide Children’s Hospital
Lai-Hua Xie: Department of Cell Biology and Molecular Medicine, New Jersey Medical School, Rutgers, The State University of New Jersey
Roger J. Hajjar: Cardiovascular Research Center, Icahn School of Medicine at Mount Sinai
Dongsheng Duan: Department of Molecular Microbiology and Immunology, Neurology, Bioengineering, Biomedical Sciences, The University of Missouri
Diego Fraidenraich: Department of Cell Biology and Molecular Medicine, New Jersey Medical School, Rutgers, The State University of New Jersey
Gopal J. Babu: Department of Cell Biology and Molecular Medicine, New Jersey Medical School, Rutgers, The State University of New Jersey

DOI: https://doi.org/10.1038/s41467-017-01146-7
Journal volume & issue: Vol. 8, no. 1
pp. 1 – 14

Abstract

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Sarcolipin is an inhibitor of the ATP dependent calcium pump SERCA, and is abnormally elevated in Duchenne muscular dystrophy. The authors show that reducing sarcolipin expression ameliorates skeletal muscle pathology and cardiomyopathy and extends life span in mouse models of DMD.

Published in Nature Communications

ISSN: 2041-1723 (Online)
Publisher: Nature Portfolio
Country of publisher: United Kingdom
LCC subjects: Science
Website: https://www.nature.com/ncomms/

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