Altered Mitochondrial Dynamic in Lymphoblasts and Fibroblasts Mutated for
Fanconi anemia
OxPhos
aerobic metabolism
fission
fusion
mitochondrial dynamics
Journal
International journal of molecular sciences
ISSN: 1422-0067
Titre abrégé: Int J Mol Sci
Pays: Switzerland
ID NLM: 101092791
Informations de publication
Date de publication:
31 Mar 2023
31 Mar 2023
Historique:
received:
19
12
2022
revised:
02
03
2023
accepted:
21
03
2023
medline:
14
4
2023
entrez:
13
4
2023
pubmed:
14
4
2023
Statut:
epublish
Résumé
Fanconi anemia (FA) is a rare genetic disorder characterized by bone marrow failure and aplastic anemia. So far, 23 genes are involved in this pathology, and their mutations lead to a defect in DNA repair. In recent years, it has been observed that FA cells also display mitochondrial metabolism defects, causing an accumulation of intracellular lipids and oxidative damage. However, the molecular mechanisms involved in the metabolic alterations have not yet been elucidated. In this work, by using lymphoblasts and fibroblasts mutated for the
Identifiants
pubmed: 37047537
pii: ijms24076557
doi: 10.3390/ijms24076557
pmc: PMC10094900
pii:
doi:
Substances chimiques
FANCA protein, human
0
Fanconi Anemia Complementation Group A Protein
0
Proteins
0
Dynamins
EC 3.6.5.5
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
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