Insufficiency of ciliary cholesterol in hereditary Zellweger syndrome.

Miyamoto, Tatsuo; Hosoba, Kosuke; Itabashi, Takeshi; Iwane, Atsuko H; Akutsu, Silvia Natsuko; Ochiai, Hiroshi; Saito, Yumiko; Yamamoto, Takashi; Matsuura, Shinya

Miyamoto, Tatsuo; Hosoba, Kosuke; Itabashi, Takeshi; Iwane, Atsuko H; Akutsu, Silvia Natsuko; Ochiai, Hiroshi; Saito, Yumiko; Yamamoto, Takashi; Matsuura, Shinya.

Afiliação

Miyamoto T; Department of Genetics and Cell Biology, Research Institute for Radiation Biology and Medicine, Hiroshima University, Hiroshima, Japan.
Hosoba K; Department of Genetics and Cell Biology, Research Institute for Radiation Biology and Medicine, Hiroshima University, Hiroshima, Japan.
Itabashi T; Program of Biomedical Science, Graduate School of Integrated Sciences for Life, Hiroshima University, Higashi-Hiroshima, Japan.
Iwane AH; Laboratory for Cell Field Structure, RIKEN Center for Biosystems Dynamics Research, Higashi-Hiroshima, Japan.
Akutsu SN; Laboratory for Cell Field Structure, RIKEN Center for Biosystems Dynamics Research, Higashi-Hiroshima, Japan.
Ochiai H; Department of Genetics and Cell Biology, Research Institute for Radiation Biology and Medicine, Hiroshima University, Hiroshima, Japan.
Saito Y; Program of Biomedical Science, Graduate School of Integrated Sciences for Life, Hiroshima University, Higashi-Hiroshima, Japan.
Yamamoto T; Program of Biomedical Science, Graduate School of Integrated Sciences for Life, Hiroshima University, Higashi-Hiroshima, Japan.
Matsuura S; Program of Life and Environmental Sciences, Graduate School of Integrated Sciences for Life, Hiroshima University, Higashi-Hiroshima, Japan.

EMBO J ; 39(12): e103499, 2020 06 17.

Article em En | MEDLINE | ID: mdl-32368833

Assuntos

Colesterol/metabolismo; Cílios/metabolismo; Síndrome de Zellweger/metabolismo; Células Cultivadas; Colesterol/genética; Cílios/genética; Cílios/patologia; Quinases do Centro Germinativo/genética; Quinases do Centro Germinativo/metabolismo; Humanos; Cinesinas/genética; Cinesinas/metabolismo; Microtúbulos/genética; Microtúbulos/metabolismo; Microtúbulos/patologia; Receptor Smoothened/genética; Receptor Smoothened/metabolismo; Síndrome de Zellweger/genética; Síndrome de Zellweger/patologia; Proteínas rab de Ligação ao GTP/genética; Proteínas rab de Ligação ao GTP/metabolismo

Palavras-chave

Zellweger syndrome; cholesterol; ciliopathy; primary cilia

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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Síndrome de Zellweger / Colesterol / Cílios Limite: Humans Idioma: En Revista: EMBO J Ano de publicação: 2020 Tipo de documento: Article País de afiliação: Japão País de publicação: Reino Unido

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