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VPS13D promotes peroxisome biogenesis.
Baldwin, Heather A; Wang, Chunxin; Kanfer, Gil; Shah, Hetal V; Velayos-Baeza, Antonio; Dulovic-Mahlow, Marija; Brüggemann, Norbert; Anding, Allyson; Baehrecke, Eric H; Maric, Dragan; Prinz, William A; Youle, Richard J.
Afiliação
  • Baldwin HA; Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD.
  • Wang C; Cell, Molecular, Developmental Biology and Biophysics Doctoral Program, Johns Hopkins University, Baltimore, MD.
  • Kanfer G; Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD.
  • Shah HV; Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD.
  • Velayos-Baeza A; Biochemistry Section, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD.
  • Dulovic-Mahlow M; Program in Neuroscience & Cognitive Science, University of Maryland, College Park, MD.
  • Brüggemann N; Wellcome Trust Centre for Human Genetics, University of Oxford, Oxford, UK.
  • Anding A; Institute of Neurogenetics, University of Lübeck, Lübeck, Germany.
  • Baehrecke EH; Institute of Neurogenetics, University of Lübeck, Lübeck, Germany.
  • Maric D; Department of Neurology, University of Lübeck, Lübeck, Germany.
  • Prinz WA; Department of Molecular, Cell and Cancer Biology, University of Massachusetts Medical School, Worcester, MA.
  • Youle RJ; Department of Molecular, Cell and Cancer Biology, University of Massachusetts Medical School, Worcester, MA.
J Cell Biol ; 220(5)2021 05 03.
Article em En | MEDLINE | ID: mdl-33891012
ABSTRACT
The VPS13 gene family consists of VPS13A-D in mammals. Although all four genes have been linked to human diseases, their cellular functions are poorly understood, particularly those of VPS13D. We generated and characterized knockouts of each VPS13 gene in HeLa cells. Among the individual knockouts, only VPS13D-KO cells exhibit abnormal mitochondrial morphology. Additionally, VPS13D loss leads to either partial or complete peroxisome loss in several transformed cell lines and in fibroblasts derived from a VPS13D mutation-carrying patient with recessive spinocerebellar ataxia. Our data show that VPS13D regulates peroxisome biogenesis.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Proteínas / Peroxissomos Idioma: En Ano de publicação: 2021 Tipo de documento: Article
Texto completo
Imprimir
XML
PubMed Links
Buscar no Google

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Proteínas / Peroxissomos Idioma: En Ano de publicação: 2021 Tipo de documento: Article