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MT001145

UniProt Annotations

Entry Information

Gene Name	ATPase family, AAA domain containing 3A
Protein Entry	ATD3A_HUMAN
UniProt ID	Q9NVI7
Species	Human

Comments

Comment type	Description
Alternative Products	Event=Alternative splicing; Named isoforms=3; Name=1; IsoId=Q9NVI7-1; Sequence=Displayed; Name=2; IsoId=Q9NVI7-2; Sequence=VSP_015636; Note=May be the predominant ATAD3A form.; Name=3; IsoId=Q9NVI7-3; Sequence=VSP_044145, VSP_015636; Note=No experimental confirmation available.;
Domain	The transmembrane domain and a C-terminal adjacent region contain all information necessary for mitochondrial targeting. {ECO:0000269\|PubMed:20154147}.
Function	Essential for mitochondrial network organization, mitochondrial metabolism and cell growth at organism and cellular level. May play an important in mitochondrial protein synthesis. May also participate in mitochondrial DNA replication. May bind to mitochondrial DNA D-loops and contribute to nucleoid stability. Required for enhanced channeling of cholesterol for hormone- dependent steroidogenesis. {ECO:0000269\|PubMed:17210950, ECO:0000269\|PubMed:20154147, ECO:0000269\|PubMed:22453275}.
Induction	Up-regulated by Angiotensin/AGT. {ECO:0000269\|PubMed:20154147, ECO:0000269\|PubMed:22453275}.
Similarity	Belongs to the AAA ATPase family. {ECO:0000305}.
Subcellular Location	Mitochondrion inner membrane; Single-pass membrane protein. Mitochondrion matrix, mitochondrion nucleoid. Note=In the mitochondrial inner membrane, enriched in sites with the potential to form contacts with the outer membrane. The N- terminal domain interacts with the inner surface of the mitochondrial outer membrane and the C-terminal domain localizes in a specific matrix compartment, where it is associated with nucleoids.
Subunit	Can form homooligomers. Homodimer formation at the N- terminus may be regulated by ATP and is required for the interaction with the inner surface of the mitochondrial outer membrane and correct mitochondrial homeostasis. Interacts with components of the mitochondrial ribosome and with other proteins involved in mitochondrial RNA metabolism. May also interact with protein involved in lipid metabolism, including STARD9. May interact with FAM210A. Interacts with GADD45GIP1. Interacts with S100B in a Ca(+2)- and Zn(+2)-dependent manner; this interaction probably occurs in the cytosol prior to mitochondrial targeting. S100B could assist ATAD3A cytoplasmic processing, preventing aggregation and favoring mitochondrial localization. Interacts with HSP60/HSPD1. Forms heterooligomers with ATAD3B; this interaction may affect ATAD3A activity. {ECO:0000269\|PubMed:20351179, ECO:0000269\|PubMed:22453275, ECO:0000269\|PubMed:22664726}.
Tissue Specificity	Overexpressed in lung adenocarcinomas (at protein level). {ECO:0000269\|PubMed:20332122}.