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Details on Person Mitochondrial isocitrate dehydrogenase IDH3 catalyzes the ir...

Class:IdSummation:71416
_displayNameMitochondrial isocitrate dehydrogenase IDH3 catalyzes the ir...
_timestamp2024-02-15 20:40:45
modified[InstanceEdit:198405] D'Eustachio, P, 2007-06-18 16:46:57
[InstanceEdit:450991] D'Eustachio, P, 2009-12-24
[InstanceEdit:1165317] D'Eustachio, P, 2010-12-14
[InstanceEdit:9853615] Stephan, Ralf, 2023-11-17
[InstanceEdit:9853653] Stephan, Ralf, 2023-11-20
[InstanceEdit:9855608] Stephan, Ralf, 2023-12-05
[InstanceEdit:9861237] D'Eustachio, Peter, 2024-02-15
textMitochondrial isocitrate dehydrogenase IDH3 catalyzes the irreversible reaction of isocitrate and NAD+ to form alpha-ketoglutarate, CO2, and NADH. The enzyme is a heterooctamer containing two copies of a heterotetramer of two IDH3A, one IDH3B, one IDH3G, and two Mn++ (PDB 7CE3; Sun et al., 2020; Dange and Colman, 2010). It is activated by ADP (Soundar et al., 2003, 2006; Bzymek and Colman, 2007) and inhibited by NADH and high concentrations of ATP (Cohen & Colman, 1972; Sun et al., 2020). This is the first of four oxidation reactions in the citric acid cycle and the first decarboxylation.

Mutations in the IDH3A, IDH3B gene can cause retinitis pigmentosa (RP46; MIM:612572; RP90; MIM:619007) (Hartong et al., 2008).
(summation)[Reaction:70967] IDH3 complex decarboxylates isocitrate [Homo sapiens]
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