Alpha-ketoglutarate dehydrogenase subunit 4 (KGD4)

Alpha-ketoglutarate dehydrogenase subunit 4 (KGD4) is an evolutionarily conserved structural subunit of the mitochondrial alpha-ketoglutarate dehydrogenase enzyme complex, crucial for the citric acid cycle and energy production in eukaryotic cells. KGD4 acts as a molecular adaptor that links the E1-E2 catalytic core to the E3 subunit, stabilizing the assembled complex and ensuring efficient enzymatic activity. Originally annotated as a mitochondrial ribosomal protein (MRPS36), further research has shown that KGD4 does not contribute to ribosomal function but rather plays a vital role in the organization and stability of the dehydrogenase complex. Defects in KGD4 are associated with mitochondrial diseases such as Leigh disease and may have broader implications in aging, neurodegeneration, and cancer due to its impact on cellular energy metabolism and mitochondrial function.

Other names

Alpha-ketoglutarate dehydrogenase component 4MRPS36DC47MRP-S3628S ribosomal protein S36, mitochondrialS36mtmitochondrial ribosomal protein S36

Not applicable (no current drugs directly target KGD4). For the alpha-ketoglutarate dehydrogenase complex: inhibition by metabolic poisons (e.g., arsenite), but these act on the enzyme complex rather than specifically on KGD4.