PARL deficiency in mouse causes Complex III defects, coenzyme Q depletion, and Leigh-like syndrome.
Animals
Brain
/ metabolism
Calcium
/ metabolism
Electron Transport Complex III
/ metabolism
Leigh Disease
/ etiology
Liver
/ metabolism
Male
Membrane Potential, Mitochondrial
Metalloproteases
/ deficiency
Mice
Mice, Knockout
Mitochondria
/ metabolism
Mitochondrial Encephalomyopathies
/ metabolism
Mitochondrial Proteins
/ deficiency
Muscle, Skeletal
/ metabolism
Reactive Oxygen Species
/ metabolism
Ubiquinone
/ metabolism
Leigh syndrome
mitochondria
neurodegeneration
respiratory chain
rhomboid protease
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:
02 01 2019
02 01 2019
Historique:
pubmed:
24
12
2018
medline:
19
3
2019
entrez:
23
12
2018
Statut:
ppublish
Résumé
The mitochondrial intramembrane rhomboid protease PARL has been implicated in diverse functions in vitro, but its physiological role in vivo remains unclear. Here we show that
Identifiants
pubmed: 30578322
pii: 1811938116
doi: 10.1073/pnas.1811938116
pmc: PMC6320509
doi:
Substances chimiques
Mitochondrial Proteins
0
Reactive Oxygen Species
0
Ubiquinone
1339-63-5
Metalloproteases
EC 3.4.-
PARL protein, mouse
EC 3.4.21.105
Electron Transport Complex III
EC 7.1.1.8
Calcium
SY7Q814VUP
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Video-Audio Media
Langues
eng
Sous-ensembles de citation
IM
Pagination
277-286Déclaration de conflit d'intérêts
The authors declare no conflict of interest.
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