Glutamate Dehydrogenase Activity Assay Kit

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

Glutamate dehydrogenase (GDH) also known as GDH1 or GLUD1 plays a critical role in ammonia metabolism by catalyzing the reversible oxidative deamination of glutamate to alpha-ketoglutarate and ammonia. This enzyme with a molecular mass of approximately 55 kDa is predominantly expressed in the liver kidney brain and pancreas. It localizes to the mitochondrial matrix indicating its importance in cellular energy production. Researchers often study GDH through various assays like the GDH assay to understand its enzymatic activities and kinetic properties.

Biological function summary

This enzyme facilitates the integration of amino acid and carbohydrate metabolism by regulating glutamate levels and influencing the tricarboxylic acid (TCA) cycle. GDH does not form part of a larger complex but acts independently. Its activity is essential for maintaining the balance between energy supply and demand in cells impacting processes such as insulin secretion in pancreatic beta-cells. Inhibitors of GDH activity like some described by Ari Meneguzzi demonstrate its importance in metabolic regulation.

Pathways

GDH functions in the ammonia detoxification and urea cycle pathways. These pathways involve the conversion of glutamate ensuring efficient nitrogen removal from the body. Key proteins linked to GDH in these pathways include carbamoyl phosphate synthetase and ornithine transcarbamylase as they collectively manage nitrogen flow. GDH also intertwines with glutamine dehydrogenase highlighting its involvement in amino acid metabolism.

Associated diseases and disorders

GDH is linked to hyperinsulinism-hyperammonemia (HI/HA) syndrome and hepatic encephalopathy. HI/HA syndrome occurs due to mutations in the GDH gene leading to inappropriate insulin secretion. Proteins such as GLUD1 are connected to GDH in the context of HI/HA. In hepatic encephalopathy altered GDH activity contributes to ammonia accumulation affecting brain function. Here GDH interactions with other mitochondrial proteins become important in disease progression.