CATALYTIC ACTIVITY : L-glutamate + H 2O + NAD(P) += 2-oxoglutarate + NH 3+ NAD(P)H. SUBUNIT : Homohexamer. SUBCELLULAR LOCATION : Mitochondrial matrix. DISEASE : OVERACTIVITY OF GLUD1 IS ASSOCIATED WITH THE HYPERINSULINISM-HYPERAMMONEMIA SYNDROME (HHS). ELEVATED OXIDATION RATE OF GLUTAMATE TO ALPHA-KETOGLUTARATE STIMULATES INSULIN SECRETION IN THE PANCREATIC BETA CELLS, WHILE THEY IMPAIRE DETOXIFICATION OF AMMONIUM IN THE LIVER. SIMILARITY : BELONGS TO THE GLU/LEU/PHE/VAL DEHYDROGENASES FAMILY. |
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19-MAR-03
Last Update 19-Mar-03