Antagonistic control of active surface integrins by myotubularin and phosphatidylinositol 3-kinase C2β in a myotubular myopathy model.


Journal

Proceedings of the National Academy of Sciences of the United States of America
ISSN: 1091-6490
Titre abrégé: Proc Natl Acad Sci U S A
Pays: United States
ID NLM: 7505876

Informations de publication

Date de publication:
04 10 2022
Historique:
entrez: 26 9 2022
pubmed: 27 9 2022
medline: 28 9 2022
Statut: ppublish

Résumé

X-linked centronuclear myopathy (XLCNM) is a severe human disease without existing therapies caused by mutations in the phosphoinositide 3-phosphatase MTM1. Loss of MTM1 function is associated with muscle fiber defects characterized by impaired localization of β-integrins and other components of focal adhesions. Here we show that defective focal adhesions and reduced active β-integrin surface levels in a cellular model of XLCNM are rescued by loss of phosphatidylinositiol 3-kinase C2β (PI3KC2β) function. Inactivation of the

Identifiants

pubmed: 36161941
doi: 10.1073/pnas.2202236119
pmc: PMC9549396
doi:

Substances chimiques

Integrins 0
Phosphatidylinositol 3-Kinase EC 2.7.1.137
Protein Tyrosine Phosphatases, Non-Receptor EC 3.1.3.48
myotubularin EC 3.1.3.48

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

e2202236119

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Auteurs

Paula Samsó (P)

Department of Molecular Pharmacology & Cell Biology, Leibniz-Forschungsinstitut für Molekulare Pharmakologie, 13125 Berlin, Germany.

Philipp A Koch (PA)

Department of Molecular Pharmacology & Cell Biology, Leibniz-Forschungsinstitut für Molekulare Pharmakologie, 13125 Berlin, Germany.

York Posor (Y)

Department of Molecular Pharmacology & Cell Biology, Leibniz-Forschungsinstitut für Molekulare Pharmakologie, 13125 Berlin, Germany.

Wen-Ting Lo (WT)

Department of Molecular Pharmacology & Cell Biology, Leibniz-Forschungsinstitut für Molekulare Pharmakologie, 13125 Berlin, Germany.

Hassane Belabed (H)

Medicinal Chemistry, Leibniz-Forschungsinstitut für Molekulare Pharmakologie, 13125 Berlin, Germany.

Marc Nazare (M)

Medicinal Chemistry, Leibniz-Forschungsinstitut für Molekulare Pharmakologie, 13125 Berlin, Germany.

Jocelyn Laporte (J)

Department of Translational Medicine and Neurogenetics, Institut de Génétique et de Biologie Moléculaire et Cellulaire, INSERM U1258, CNRS UMR7104, Strasbourg University, 67404 Illkirch, France.

Volker Haucke (V)

Department of Molecular Pharmacology & Cell Biology, Leibniz-Forschungsinstitut für Molekulare Pharmakologie, 13125 Berlin, Germany.
Faculty of Biology, Chemistry and Pharmacy, Freie Universität Berlin, 14195 Berlin, Germany.

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Classifications MeSH