2-OG

2-Oxoglutarate (also known as 2-OG) is a key metabolic intermediate that plays a significant role in various biochemical pathways. It is a product of the TCA cycle, also known as the Krebs cycle or the citric acid cycle, and is involved in amino acid metabolism, lipid metabolism, and the urea cycle.

Biochemical Role

2-OG is a key intermediate in the TCA cycle, where it is converted to succinyl-CoA by the enzyme alpha-ketoglutarate dehydrogenase. This reaction is a key step in the cycle and is highly regulated.

In addition to its role in the TCA cycle, 2-OG is also involved in amino acid metabolism. It serves as a substrate for the enzyme glutamate dehydrogenase, which converts it to glutamate, an important neurotransmitter.

2-OG also plays a role in lipid metabolism, where it is involved in the synthesis of fatty acids and cholesterol.

Finally, 2-OG is involved in the urea cycle, where it is converted to ornithine by the enzyme ornithine transcarbamylase. This reaction is a key step in the cycle, which is responsible for the removal of ammonia from the body.

Clinical Significance

Abnormalities in the metabolism of 2-OG can lead to various health conditions. For example, deficiencies in the enzyme alpha-ketoglutarate dehydrogenase can lead to neurological disorders such as Alzheimer's disease and Parkinson's disease.

Furthermore, mutations in the gene encoding for the enzyme ornithine transcarbamylase can lead to ornithine transcarbamylase deficiency, a rare genetic disorder that affects the body's ability to remove ammonia from the body.

See Also

• TCA cycle
• Amino acid metabolism
• Lipid metabolism
• Urea cycle

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