Muscle-specific lack of Gfpt1 triggers ER stress to alleviate misfolded protein accumulation.

Zhang, Ruchen; Farshadyeganeh, Paniz; Ohkawara, Bisei; Nakajima, Kazuki; Takeda, Jun-Ichi; Ito, Mikako; Zhang, Shaochuan; Miyasaka, Yuki; Ohno, Tamio; Mori-Yoshimura, Madoka; Masuda, Akio; Ohno, Kinji

Zhang, Ruchen; Farshadyeganeh, Paniz; Ohkawara, Bisei; Nakajima, Kazuki; Takeda, Jun-Ichi; Ito, Mikako; Zhang, Shaochuan; Miyasaka, Yuki; Ohno, Tamio; Mori-Yoshimura, Madoka; Masuda, Akio; Ohno, Kinji.

Afiliação

Zhang R; Division of Neurogenetics, Center for Neurological Diseases and Cancer, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan.
Farshadyeganeh P; Division of Neurogenetics, Center for Neurological Diseases and Cancer, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan.
Ohkawara B; Division of Neurogenetics, Center for Neurological Diseases and Cancer, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan.
Nakajima K; Institute for Glyco-core Research (iGCORE), Gifu University, Gifu 501-1193, Japan.
Takeda JI; Division of Neurogenetics, Center for Neurological Diseases and Cancer, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan.
Ito M; Division of Neurogenetics, Center for Neurological Diseases and Cancer, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan.
Zhang S; Division of Neurogenetics, Center for Neurological Diseases and Cancer, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan.
Miyasaka Y; Division of Experimental Animals, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan.
Ohno T; Division of Experimental Animals, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan.
Mori-Yoshimura M; Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Kodaira 187-8775, Japan.
Masuda A; Division of Neurogenetics, Center for Neurological Diseases and Cancer, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan.
Ohno K; Division of Neurogenetics, Center for Neurological Diseases and Cancer, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan.

Dis Model Mech ; 17(8)2024 Aug 01.

Article em En | MEDLINE | ID: mdl-38903011

Assuntos

Autofagia; Estresse do Retículo Endoplasmático; Músculo Esquelético; Dobramento de Proteína; Resposta a Proteínas não Dobradas; Animais; Camundongos; Apoptose; Proteína Forkhead Box O1/metabolismo; Técnicas de Introdução de Genes; Fibras Musculares Esqueléticas/metabolismo; Fibras Musculares Esqueléticas/patologia; Músculo Esquelético/metabolismo; Músculo Esquelético/patologia; Junção Neuromuscular/metabolismo; Junção Neuromuscular/patologia; Especificidade de Órgãos; Complexo de Endopeptidases do Proteassoma/metabolismo; Agregados Proteicos; Proteína 1 de Ligação a X-Box/metabolismo

Palavras-chave

Congenital myasthenic syndrome (CMS); Endoplasmic reticulum (ER) stress; Glutamine fructose-6-phosphate transaminase 1 (GFPT1); Hexosamine biosynthesis pathway (HBP); Neuromuscular junction (NMJ); Unfolded protein response (UPR)

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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Autofagia / Dobramento de Proteína / Músculo Esquelético / Resposta a Proteínas não Dobradas / Estresse do Retículo Endoplasmático Limite: Animals Idioma: En Ano de publicação: 2024 Tipo de documento: Article

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