Metabolic switch from fatty acid oxidation to glycolysis in knock-in mouse model of Barth syndrome.
Barth syndrome
cardiolipin
cardiomyopathy
mitochondria
tafazzin
Journal
EMBO molecular medicine
ISSN: 1757-4684
Titre abrégé: EMBO Mol Med
Pays: England
ID NLM: 101487380
Informations de publication
Date de publication:
11 09 2023
11 09 2023
Historique:
revised:
18
07
2023
received:
06
01
2023
accepted:
19
07
2023
medline:
12
9
2023
pubmed:
3
8
2023
entrez:
3
8
2023
Statut:
ppublish
Résumé
Mitochondria are central for cellular metabolism and energy supply. Barth syndrome (BTHS) is a severe disorder, due to dysfunction of the mitochondrial cardiolipin acyl transferase tafazzin. Altered cardiolipin remodeling affects mitochondrial inner membrane organization and function of membrane proteins such as transporters and the oxidative phosphorylation (OXPHOS) system. Here, we describe a mouse model that carries a G197V exchange in tafazzin, corresponding to BTHS patients. TAZ
Identifiants
pubmed: 37533404
doi: 10.15252/emmm.202317399
pmc: PMC10493589
doi:
Substances chimiques
Cardiolipins
0
AMP-Activated Protein Kinases
EC 2.7.11.31
Fatty Acids
0
Adenosine Triphosphate
8L70Q75FXE
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
e17399Informations de copyright
© 2023 The Authors. Published under the terms of the CC BY 4.0 license.
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