Truncated dystrophin ameliorates the dystrophic phenotype of mdx mice by reducing sarcolipin-mediated SERCA inhibition.

Tanihata, Jun; Nagata, Tetsuya; Ito, Naoki; Saito, Takashi; Nakamura, Akinori; Minamisawa, Susumu; Aoki, Yoshitsugu; Ruegg, Urs T; Takeda, Shin'ichi

Tanihata, Jun; Nagata, Tetsuya; Ito, Naoki; Saito, Takashi; Nakamura, Akinori; Minamisawa, Susumu; Aoki, Yoshitsugu; Ruegg, Urs T; Takeda, Shin'ichi.

Afiliação

Tanihata J; Department of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP), Kodaira, Japan; Department of Cell Physiology, The Jikei University School of Medicine, Minato, Japan.
Nagata T; Department of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP), Kodaira, Japan.
Ito N; Department of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP), Kodaira, Japan.
Saito T; Department of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP), Kodaira, Japan.
Nakamura A; Third Department of Internal Medicine, Shinshu University School of Medicine, Matsumoto, Japan.
Minamisawa S; Department of Cell Physiology, The Jikei University School of Medicine, Minato, Japan.
Aoki Y; Department of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP), Kodaira, Japan.
Ruegg UT; Pharmacology, Geneva-Lausanne School of Pharmaceutical Sciences, University of Geneva, Geneva, Switzerland.
Takeda S; Department of Molecular Therapy, National Institute of Neuroscience, National Center of Neurology and Psychiatry (NCNP), Kodaira, Japan. Electronic address: takeda@ncnp.go.jp.

Biochem Biophys Res Commun ; 505(1): 51-59, 2018 10 20.

Article em En | MEDLINE | ID: mdl-30236982

Assuntos

Distrofina/metabolismo; Proteínas Musculares/metabolismo; Distrofia Muscular de Duchenne/metabolismo; Proteolipídeos/metabolismo; ATPases Transportadoras de Cálcio do Retículo Sarcoplasmático/metabolismo; Animais; Cálcio/metabolismo; Células Cultivadas; Distrofina/genética; Humanos; Camundongos Endogâmicos C57BL; Camundongos Endogâmicos mdx; Camundongos Knockout; Camundongos Transgênicos; Proteínas Musculares/genética; Distrofia Muscular de Duchenne/genética; Distrofia Muscular de Duchenne/patologia; Óxido Nítrico Sintase Tipo I/genética; Óxido Nítrico Sintase Tipo I/metabolismo; Fenótipo; Proteolipídeos/genética; ATPases Transportadoras de Cálcio do Retículo Sarcoplasmático/genética; Transgenes/genética

Palavras-chave

Becker muscular dystrophy; Duchenne muscular dystrophy; Ryanodine receptor; Sarcolipin; Sarcoplasmic/endoplasmic reticulum Ca(2+)-ATPase (SERCA); nNOS

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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Proteolipídeos / Distrofina / Distrofia Muscular de Duchenne / ATPases Transportadoras de Cálcio do Retículo Sarcoplasmático / Proteínas Musculares Limite: Animals / Humans Idioma: En Revista: Biochem Biophys Res Commun Ano de publicação: 2018 Tipo de documento: Article País de afiliação: Japão

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