ATPIF1

ATPIF1
Identifiers
Aliases	ATPIF1, ATPI, ATPIP, IP, ATPase inhibitory factor 1
External IDs	MGI: 1196457 HomoloGene: 40581 GeneCards: ATPIF1
Gene ontology Molecular function • 5-formyltetrahydrofolate cyclo-ligase activity • enzyme inhibitor activity • protein homodimerization activity • ATPase binding • calmodulin binding • ATPase inhibitor activity • angiostatin binding Cellular component • mitochondrial proton-transporting ATP synthase complex • cell surface • mitochondrion Biological process • regulation of ATP metabolic process • mitophagy in response to mitochondrial depolarization • positive regulation of mitochondrial outer membrane permeabilization involved in apoptotic signaling pathway • negative regulation of ATPase activity • tetrahydrofolate interconversion • positive regulation of proteolysis involved in cellular protein catabolic process • negative regulation of hydrolase activity • generation of precursor metabolites and energy • reactive oxygen species metabolic process • negative regulation of endothelial cell proliferation • heme biosynthetic process • regulation of protein targeting to mitochondrion • angiogenesis • erythrocyte differentiation • mitochondrial depolarization • protein homooligomerization • protein homotetramerization • folic acid-containing compound biosynthetic process Sources:Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	93974	11983
Ensembl	ENSG00000130770	ENSMUSG00000054428
UniProt	Q9UII2	O35143
RefSeq (mRNA)	NM_178191 NM_016311 NM_178190	NM_007512
RefSeq (protein)	NP_057395.1 NP_835497.1 NP_835498.1	NP_031538.2
Location (UCSC)	Chr 1: 28.24 – 28.25 Mb	Chr 4: 132.53 – 132.53 Mb
PubMed search	[1]	[2]
Wikidata

ATPase inhibitor, mitochondrial is an enzyme that in humans is encoded by the ATPIF1 gene.^[3][4]

This gene encodes a mitochondrial ATPase inhibitor. Alternative splicing occurs at this locus and three transcript variants encoding distinct isoforms have been identified.^[4]

It prevents ATPase from switching to ATP hydrolysis during collapse of the electrochemical gradient, for example during oxygen deprivation ^[5] ATP synthase inhibitor forms a one-to-one complex with the F1 ATPase, possibly by binding at the alpha-beta interface. It is thought to inhibit ATP synthesis by preventing the release of ATP.^[6] The inhibitor has two oligomeric states, dimer (the active state) and tetramer. At low pH, the inhibitor forms a dimer via antiparallel coiled coil interactions between the C-terminal regions of two monomers. At high pH, the inhibitor forms tetramers and higher oligomers by coiled coil interactions involving the N terminus and inhibitory region, thus preventing the inhibitory activity.^[5]

Model organisms

Model organisms have been used in the study of ATPIF1 function. A conditional knockout mouse line, called Atpif1^{tm1a(EUCOMM)Wtsi[13][14]} was generated as part of the International Knockout Mouse Consortium program — a high-throughput mutagenesis project to generate and distribute animal models of disease to interested scientists.^[15][16][17]

Male and female animals underwent a standardized phenotypic screen to determine the effects of deletion.^[11][18] Twenty three tests were carried out on mutant mice and three significant abnormalities were observed.^[11] Homozygous mutant animals displayed hyperactivity and brain dysmorphology, while males also had decreased circulating alkaline phosphatase levels.^[11]

Mitochondrial ATPase inhibitor, IATP
c-terminal coiled-coil domain from bovine if1
Identifiers
Symbol	IATP
Pfam	PF04568
InterPro	IPR007648
SCOP	1hf9
SUPERFAMILY	1hf9
Available protein structures: Pfam structures PDB RCSB PDB; PDBe; PDBj PDBsum structure summary

References

Further reading

This article incorporates text from the public domain Pfam and InterPro IPR007648