Recombinant Human L2HGDH protein

Supplementary info

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

Activity summary

L2HGDH also known as L-2-hydroxyglutarate dehydrogenase is an enzyme that plays an important role in cellular metabolism. It catalyzes the conversion of L-2-hydroxyglutarate to alpha-ketoglutarate an important step in metabolizing alpha-hydroxy acids. The protein has an approximate molecular weight of 51 kDa. L2HGDH is predominantly expressed in the liver kidney and brain tissues where metabolic activities frequently occur.

Biological function summary

This enzyme participates in the detoxification process of L-2-hydroxyglutarate within mitochondria. L2HGDH functions independently rather than as part of a multicellular complex. The activity of this enzyme ensures that L-2-hydroxyglutarate does not accumulate which would otherwise interfere with normal cellular processes and lead to dysfunctions.

Pathways

The enzymatic action of L2HGDH integrates into the alpha-ketoglutarate metabolism and the larger tricarboxylic acid (TCA) cycle. It forms part of the process that prevents the buildup of L-2-hydroxyglutarate. Alpha-ketoglutarate which L2HGDH helps generate is an important component of the TCA cycle and therefore affects energy production within the cell. The activity of the related protein IDH2 is important as both enzymes contribute to maintaining normal levels of 2-hydroxyglutarate isomers within the cell.

Associated diseases and disorders

Impaired function of L2HGDH can lead to L-2-hydroxyglutaric aciduria a rare neurometabolic disorder. This disorder is characterized by the accumulation of L-2-hydroxyglutarate resulting in progressive neurological symptoms. Additionally research indicates connections between L2HGDH malfunction and certain cancers owing to disrupted metabolic pathways. In some cancers mutations in L2HGDH or its related pathways involve IDH1 and IDH2 proteins which increase oncometabolite levels that promote tumorigenesis.