Structural instability of lamin A tail domain modulates its assembly and higher order function in Emery-Dreifuss muscular dystrophy.

Mio, Muneyo; Sugiki, Toshihiko; Matsuda, Chie; Mitsuhashi, Hiroaki; Kojima, Chojiro; Chan, Siu Yuen; Hayashi, Yukiko K; Mio, Kazuhiro

Mio, Muneyo; Sugiki, Toshihiko; Matsuda, Chie; Mitsuhashi, Hiroaki; Kojima, Chojiro; Chan, Siu Yuen; Hayashi, Yukiko K; Mio, Kazuhiro.

Afiliação

Mio M; Molecular Profiling Research Center for Drug Discovery and OPERANDO Open Innovation Laboratory, National Institute of Advanced Industrial Science and Technology (AIST), Tokyo, 135-0064, Japan; Graduate School of Medical Life Science, Yokohama City University, Kanagawa, 230-0045, Japan.
Sugiki T; Institute for Protein Research, Osaka University, Osaka, 565-0871, Japan.
Matsuda C; Biomedical Research Institute, National Institute of Advanced Industrial Science and Technology (AIST), Ibaraki, 305-8566, Japan.
Mitsuhashi H; Department of Applied Biochemistry, School of Engineering, Tokai University, Kanagawa, 259-1207, Japan.
Kojima C; Institute for Protein Research, Osaka University, Osaka, 565-0871, Japan; Graduate School of Engineering Science, Yokohama National University, Kanagawa, 240-8501, Japan.
Chan SY; Department of Paediatrics and Adolescent Medicine, Li Ka Shing Faculty of Medicine, The University of Hong Kong, Hong Kong, China.
Hayashi YK; Department of Pathophysiology, Tokyo Medical University, Tokyo, 160-8402, Japan. Electronic address: yhayashi@tokyo-med.ac.jp.
Mio K; Molecular Profiling Research Center for Drug Discovery and OPERANDO Open Innovation Laboratory, National Institute of Advanced Industrial Science and Technology (AIST), Tokyo, 135-0064, Japan; Graduate School of Medical Life Science, Yokohama City University, Kanagawa, 230-0045, Japan. Electronic ad

Biochem Biophys Res Commun ; 512(1): 22-28, 2019 04 23.

Article em En | MEDLINE | ID: mdl-30853177

Assuntos

Lamina Tipo A/química; Distrofia Muscular de Emery-Dreifuss/metabolismo; Substituição de Aminoácidos; Fenômenos Biofísicos; DNA/química; DNA/metabolismo; Humanos; Técnicas In Vitro; Lamina Tipo A/genética; Lamina Tipo A/metabolismo; Lipodistrofia Parcial Familiar/genética; Lipodistrofia Parcial Familiar/metabolismo; Microscopia Eletrônica de Transmissão; Modelos Moleculares; Distrofia Muscular de Emery-Dreifuss/genética; Mutação; Fragmentos de Peptídeos/química; Fragmentos de Peptídeos/genética; Fragmentos de Peptídeos/metabolismo; Domínios Proteicos; Estabilidade Proteica; Proteínas Recombinantes/química; Proteínas Recombinantes/genética; Proteínas Recombinantes/metabolismo

Palavras-chave

Ig-domain; Lamin A; Laminopathy; Thermal stability

Texto completo

Imprimir

XML

PubMed Links

Buscar no Google

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Distrofia Muscular de Emery-Dreifuss / Lamina Tipo A Limite: Humans Idioma: En Ano de publicação: 2019 Tipo de documento: Article

Texto completo

Imprimir

XML

PubMed Links

Buscar no Google