IDH3B


Description

The IDH3B (isocitrate dehydrogenase (NAD(+)) 3 non-catalytic subunit beta) is a protein-coding gene located on chromosome 20.

The IDH3B gene encodes the beta subunit of the NAD(+)-dependent isocitrate dehydrogenase enzyme (IDH3) in humans. IDH3 is a mitochondrial enzyme that plays a critical role in the tricarboxylic acid cycle (TCA cycle) by catalyzing the oxidative decarboxylation of isocitrate to 2-oxoglutarate. The enzyme is a heterotetramer composed of two alpha subunits (IDH3A), one beta subunit (IDH3B), and one gamma subunit (IDH3G). The IDH3B protein contributes to the assembly and full activity of the enzyme, ensuring efficient catalysis of the critical step in the TCA cycle.

IDH3B plays a crucial structural role in the assembly and full activity of the isocitrate dehydrogenase enzyme (IDH3). It forms a heterodimer with the alpha subunit (IDH3A), contributing to the enzyme's basal activity. However, the full catalytic activity of IDH3 requires the assembly of two heterodimers, one containing IDH3A and IDH3B, and the other containing IDH3A and IDH3G, into a heterotetramer. This assembly and cooperative function of both heterodimers are essential for the enzyme to efficiently catalyze the decarboxylation of isocitrate (ICT) into alpha-ketoglutarate.

IDH3B is also known as RP46.

Associated Diseases


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