Catalyzes the first reaction in the primary pathway for the renal catabolism of glutamine. Plays a role in maintaining acid-base homeostasis. Regulates the levels of the neurotransmitter glutamate, the main excitatory neurotransmitter in the brain ( ).
Lacks catalytic activity.
Subcellular locations: 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.
Subcellular locations: Mitochondrion
Subcellular locations: Mitochondrion matrix
Produced by the proteolytic processing of the 74-kDa cytosolic precursor.
Subcellular locations: Mitochondrion matrix
Produced by the proteolytic processing of the 74-kDa cytosolic precursor.
Isoform 1 and isoform 3 are detected in brain cortex. Isoform 3 is highly expressed in astrocytoma, ganglioglioma and ependymoma. Isoform 1 is highly expressed in brain and kidney, but not detected in liver. Isoform 3 is highly expressed in heart and pancreas, detected at lower levels in placenta, lung, pancreas and kidney, but is not detected in liver. Isoform 2 is expressed in cardiac and skeletal muscle.