ATPIF1

ATPase inhibitor, mitochondrial is an is_associated_with::enzyme that in humans is encoded by the ATPIF1 is_associated_with::gene.

It prevents is_associated_with::ATPase from switching to ATP is_associated_with::hydrolysis during collapse of the electrochemical gradient, for example during is_associated_with::oxygen deprivation 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. The inhibitor has two oligomeric states, dimer (the active state) and is_associated_with::tetramer. At low is_associated_with::pH, the inhibitor forms a dimer via antiparallel is_associated_with::coiled coil interactions between the is_associated_with::C-terminal regions of two is_associated_with::monomers. At high pH, the inhibitor forms tetramers and higher is_associated_with::oligomers by coiled coil interactions involving the is_associated_with::N terminus and inhibitory region, thus preventing the inhibitory activity.

Model organisms
is_associated_with::Model organisms have been used in the study of ATPIF1 function. A conditional is_associated_with::knockout mouse line, called Atpif1tm1a(EUCOMM)Wtsi was generated as part of the is_associated_with::International Knockout Mouse Consortium program — a high-throughput mutagenesis project to generate and distribute animal models of disease to interested scientists.

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