ATAD3A has a scaffolding role regulating mitochondria inner membrane structure and protein assembly.
ATPases Associated with Diverse Cellular Activities
/ genetics
Animals
Brain Diseases
/ metabolism
Female
HEK293 Cells
Humans
Male
Membrane Proteins
/ genetics
Mice
Mice, Inbred C57BL
Mice, Knockout
Mitochondria
/ metabolism
Mitochondrial Membranes
/ metabolism
Mitochondrial Proteins
/ genetics
Neurons
/ metabolism
Oxidative Phosphorylation
Sequence Deletion
Transcriptome
ATAD3
cardiolipin
cristae
inner membrane
mitochondria
Journal
Cell reports
ISSN: 2211-1247
Titre abrégé: Cell Rep
Pays: United States
ID NLM: 101573691
Informations de publication
Date de publication:
21 12 2021
21 12 2021
Historique:
received:
31
03
2021
revised:
02
09
2021
accepted:
24
11
2021
entrez:
22
12
2021
pubmed:
23
12
2021
medline:
16
2
2022
Statut:
ppublish
Résumé
The ATPase Family AAA Domain Containing 3A (ATAD3A), is a mitochondrial inner membrane protein conserved in metazoans. ATAD3A has been associated with several mitochondrial functions, including nucleoid organization, cholesterol metabolism, and mitochondrial translation. To address its primary role, we generated a neuronal-specific conditional knockout (Atad3 nKO) mouse model, which developed a severe encephalopathy by 5 months of age. Pre-symptomatic mice showed aberrant mitochondrial cristae morphogenesis in the cortex as early as 2 months. Using a multi-omics approach in the CNS of 2-to-3-month-old mice, we found early alterations in the organelle membrane structure. We also show that human ATAD3A associates with different components of the inner membrane, including OXPHOS complex I, Letm1, and prohibitin complexes. Stochastic Optical Reconstruction Microscopy (STORM) shows that ATAD3A is regularly distributed along the inner mitochondrial membrane, suggesting a critical structural role in inner mitochondrial membrane and its organization, most likely in an ATPase-dependent manner.
Identifiants
pubmed: 34936866
pii: S2211-1247(21)01635-1
doi: 10.1016/j.celrep.2021.110139
pmc: PMC8785211
mid: NIHMS1766439
pii:
doi:
Substances chimiques
ATAD3A protein, human
0
Atad3a protein, mouse
0
Membrane Proteins
0
Mitochondrial Proteins
0
ATPases Associated with Diverse Cellular Activities
EC 3.6.4.-
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Langues
eng
Sous-ensembles de citation
IM
Pagination
110139Subventions
Organisme : NEI NIH HHS
ID : R01 EY010804
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS079965
Pays : United States
Organisme : NIGMS NIH HHS
ID : R35 GM118141
Pays : United States
Organisme : NIA NIH HHS
ID : R01 AG036871
Pays : United States
Organisme : NIEHS NIH HHS
ID : R33 ES025673
Pays : United States
Informations de copyright
Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.
Déclaration de conflit d'intérêts
Declaration of interests The authors declare no conflict of interests.
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