Glutamate Dehydrogenase 1 (GDH) ELISA Kit for Chicken

This gene encodes glutamate dehydrogenase protein; a mitochondrial matrix enzyme that catalyzes the oxidative deamination of glutamate to alpha-ketoglutarate and ammonia. This enzyme has an important role in regulating amino acid induced insulin secretion and activating mutations in this gene are a common cause of congenital hyperinsulinism. This enzyme is allosterically activated by ADP and inhibited by GTP and ATP. The related glutamate dehydrogenase 2 gene on the human X-chromosome originated from this gene via retrotransposition and encodes a soluble form of glutamate dehydrogenase. Multiple pseudogenes of this gene are present in humans.[provided by RefSeq, Sep 2009]

GLUD1: May be involved in learning and memory reactions by increasing the turnover of the excitatory neurotransmitter glutamate. Homohexamer. Subject to allosteric regulation. Activated by ADP. Inhibited by GTP and ATP. ADP can occupy the NADH binding site and activate the enzyme. Belongs to the Glu/Leu/Phe/Val dehydrogenases family.

Protein type: EC 1.4.1.3; Energy Metabolism - nitrogen; Amino Acid Metabolism - arginine and proline; Mitochondrial; Amino Acid Metabolism - alanine, aspartate and glutamate; Oxidoreductase; Other Amino Acids Metabolism - D-Glutamine and D-glutamate

Chromosomal Location of Human Ortholog: 10q23.3

Cellular Component: mitochondrion; mitochondrial matrix; cytoplasm

Molecular Function: identical protein binding; protein binding; glutamate dehydrogenase activity; glutamate dehydrogenase [NAD(P)+] activity; GTP binding; ADP binding; ATP binding

Biological Process: glutamate catabolic process; substantia nigra development; positive regulation of insulin secretion; glutamine metabolic process; glutamate biosynthetic process; amino acid biosynthetic process

Disease: Hyperinsulinemic Hypoglycemia, Familial, 6