Oxalosuccinic acid

Oxalosuccinic acid is an intermediate compound in the citric acid cycle, also known as the Krebs cycle or the tricarboxylic acid cycle. It is formed from isocitrate via the enzyme isocitrate dehydrogenase, and is subsequently converted into alpha-ketoglutarate.

Structure and Properties

Oxalosuccinic acid is a dicarboxylic acid with the chemical formula C4H4O7. It contains two carboxyl groups (-COOH) and one keto group (=O). The molecule is highly polar due to the presence of these functional groups, and it is therefore soluble in water.

Role in the Citric Acid Cycle

In the citric acid cycle, oxalosuccinic acid plays a crucial role in the conversion of isocitrate to alpha-ketoglutarate. This reaction is catalyzed by the enzyme isocitrate dehydrogenase, which removes a hydrogen atom from isocitrate and adds a carbonyl group to form oxalosuccinic acid. The oxalosuccinic acid is then decarboxylated to form alpha-ketoglutarate, releasing a molecule of carbon dioxide in the process.

Clinical Significance

Abnormalities in the metabolism of oxalosuccinic acid can lead to various health conditions. For example, a deficiency in isocitrate dehydrogenase can lead to an accumulation of isocitrate and a decrease in the levels of alpha-ketoglutarate and other downstream metabolites, potentially leading to metabolic acidosis and other metabolic disorders.

See Also

• Citric acid cycle
• Isocitrate dehydrogenase
• Alpha-ketoglutarate

References

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