This gene encodes the K-type mitochondrial glutaminase. The encoded protein is an phosphate-activated amidohydrolase that catalyzes the hydrolysis of glutamine to glutamate and ammonia. This protein is primarily expressed in the brain and kidney plays an essential role in generating energy for metabolism, synthesizing the brain neurotransmitter glutamate and maintaining acid-base balance in the kidney. Alternate splicing results in multiple transcript variants. [provided by RefSeq, Jan 2012],
show all
Function:
Catalytic activity:L-glutamine + H(2)O = L-glutamate + NH(3).,Function:Catalyzes the first reaction in the primary pathway for the renal catabolism of glutamine.,similarity:Belongs to the glutaminase family.,similarity:Contains 1 ANK repeat.,tissue specificity:KGA is expressed predominantly in brain and kidney but not in liver, GAC is expressed principally in cardiac muscle and pancreas but not in liver or brain, and GAM is expressed solely in cardiac and skeletal muscle.,
show all
Cellular Localization:
[Isoform 1]: Mitochondrion . Cytoplasm, cytosol . The 74-kDa cytosolic precursor is translocated into the mitochondria and processed via a 72-kDa intermediate to yield the mature 68- and 65-kDa subunits. .; [Isoform 3]: Mitochondrion .; [Glutaminase kidney isoform, mitochondrial 68 kDa chain]: Mitochondrion matrix . Produced by the proteolytic processing of the 74-kDa cytosolic precursor. .; [Glutaminase kidney isoform, mitochondrial 65 kDa chain]: Mitochondrion matrix . Produced by the proteolytic processing of the 74-kDa cytosolic precursor. .