ANT-dependent MPTP underlies necrotic myofiber death in muscular dystrophy.

Animals Mice Muscular Dystrophies Necrosis Cell Death Peptidyl-Prolyl Isomerase F Disease Models, Animal

Journal

Science advances
ISSN: 2375-2548
Titre abrégé: Sci Adv
Pays: United States
ID NLM: 101653440

Informations de publication

Date de publication:
25 08 2023
Historique:
medline: 28 8 2023
pubmed: 25 8 2023
entrez: 25 8 2023
Statut: ppublish

Résumé

Mitochondrial permeability transition pore (MPTP) formation contributes to ischemia-reperfusion injury in the heart and several degenerative diseases, including muscular dystrophy (MD). MD is a family of genetic disorders characterized by progressive muscle necrosis and premature death. It has been proposed that the MPTP has two molecular components, the adenine nucleotide translocase (ANT) family of proteins and an unknown component that requires the chaperone cyclophilin D (CypD) to activate. This model was examined in vivo by deleting the gene encoding ANT1 (

Identifiants

DOI: 10.1126/sciadv.adi2767 PMID: 37624892 PMC: PMC10456852
pubmed: 37624892
doi: 10.1126/sciadv.adi2767
pmc: PMC10456852
doi:

Substances chimiques

Peptidyl-Prolyl Isomerase F 0

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

eadi2767

Subventions

Organisme : NIAMS NIH HHS
ID : R01 AR071301
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL132831
Pays : United States

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Auteurs

Michael J Bround (MJ)

Department of Pediatrics, Cincinnati Children's Hospital and the University of Cincinnati, Cincinnati, OH, USA.
ORCID: 0000-0003-3238-5489

Julian R Havens (JR)

Department of Pediatrics, Cincinnati Children's Hospital and the University of Cincinnati, Cincinnati, OH, USA.
ORCID: 0000-0001-6242-0965

Allen J York (AJ)

Department of Pediatrics, Cincinnati Children's Hospital and the University of Cincinnati, Cincinnati, OH, USA.
ORCID: 0000-0002-3174-0306

Michelle A Sargent (MA)

Department of Pediatrics, Cincinnati Children's Hospital and the University of Cincinnati, Cincinnati, OH, USA.

Jason Karch (J)

Department of Integrative Physiology, Baylor College of Medicine, Houston, TX, USA.
ORCID: 0000-0002-3023-7469

Jeffery D Molkentin (JD)

Department of Pediatrics, Cincinnati Children's Hospital and the University of Cincinnati, Cincinnati, OH, USA.
ORCID: 0000-0002-3558-6529

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Classifications MeSH

questionsmedicales.fr › Eucaryotes › Animaux › ANT-dependent MPTP underlies necrotic myofiber...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Rodentia › Muridae › Murinae › Souris › ANT-dependent MPTP underlies necrotic myofiber...
questionsmedicales.fr › Malformations et maladies congénitales, héréditaires et néonatales › Maladies génétiques congénitales › Dystrophies musculaires › ANT-dependent MPTP underlies necrotic myofiber...
questionsmedicales.fr › États, signes et symptômes pathologiques › Processus pathologiques › Nécrose › ANT-dependent MPTP underlies necrotic myofiber...
questionsmedicales.fr › Phénomènes physiologiques cellulaires › Mort cellulaire › ANT-dependent MPTP underlies necrotic myofiber...
questionsmedicales.fr › Techniques d'investigation › Modèles théoriques › Modèles biologiques › Modèles animaux de maladie humaine › ANT-dependent MPTP underlies necrotic myofiber...