GOT2
Glutamic-oxaloacetic transaminase is a pyridoxal phosphate-dependent enzyme which exists in cytoplasmic and inner-membrane mitochondrial forms, GOT1 and GOT2, respectively. GOT plays a role in amino acid metabolism and the urea and tricarboxylic acid cycles. The two enzymes are homodimeric and show close homology. Two transcript variants encoding different isoforms have been found for this gene.
Full Name
glutamic-oxaloacetic transaminase 2
Function
Catalyzes the irreversible transamination of the L-tryptophan metabolite L-kynurenine to form kynurenic acid (KA). As a member of the malate-aspartate shuttle, it has a key role in the intracellular NAD(H) redox balance. Is important for metabolite exchange between mitochondria and cytosol, and for amino acid metabolism. Facilitates cellular uptake of long-chain free fatty acids.
Biological Process
2-oxoglutarate metabolic process Source: UniProtKB
4-hydroxyproline catabolic process Source: BHF-UCL
Aspartate biosynthetic process Source: Ensembl
Aspartate catabolic process Source: UniProtKB
Aspartate metabolic process Source: UniProtKB
Fatty acid transport Source: UniProtKB
Female pregnancy Source: Ensembl
Glutamate catabolic process to 2-oxoglutarate Source: Ensembl
Glutamate catabolic process to aspartate Source: Ensembl
Glutamate metabolic process Source: UniProtKB
Lactation Source: Ensembl
Oxaloacetate metabolic process Source: Ensembl
Response to ethanol Source: UniProtKB
Response to insulin Source: Ensembl
Response to morphine Source: Ensembl
Response to muscle activity Source: Ensembl
Cellular Location
Mitochondrion matrix; Cell membrane. Exposure to alcohol promotes translocation to the cell membrane.
Involvement in disease
Developmental and epileptic encephalopathy 82 (DEE82):
A form of epileptic encephalopathy, a heterogeneous group of severe early-onset epilepsies characterized by refractory seizures, neurodevelopmental impairment, and poor prognosis. Development is normal prior to seizure onset, after which cognitive and motor delays become apparent. DEE82 is an autosomal recessive metabolic encephalopathy characterized by epilepsy from the first year of life, global developmental delay, hypotonia and feeding difficulties apparent soon after birth, and intellectual and motor disabilities. Other features include poor overall growth, progressive microcephaly and biochemical abnormalities, including increased serum lactate and ammonia.
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