SDHB

SDHB or Succinate dehydrogenase complex iron sulfur subunit B is a protein that in humans is encoded by the SDHB gene. It is located on the short (p) arm of chromosome 1 at position 36.1. The protein encoded by this gene is a subunit of the succinate dehydrogenase (SDH) complex, a key enzyme complex of the tricarboxylic acid cycle (TCA cycle) and the electron transport chain.

Function

The SDHB gene provides instructions for making one part of the succinate dehydrogenase complex. This complex is embedded in the inner mitochondrial membrane, where it participates in both the tricarboxylic acid cycle and the electron transport chain. These processes are critical for converting glucose and other nutrients into adenosine triphosphate (ATP), the cell's main energy source.

Clinical significance

Mutations in the SDHB gene are associated with hereditary paraganglioma-pheochromocytoma syndromes. These conditions increase the risk of developing noncancerous (benign) tumors in the adrenal glands and other parts of the body. The SDHB gene mutations that cause these conditions lead to the production of an abnormal version of the SDHB protein that cannot properly function in the succinate dehydrogenase complex.

See also

• Succinate dehydrogenase
• Tricarboxylic acid cycle
• Electron transport chain
• Mitochondrial membrane
• Adenosine triphosphate
• Hereditary paraganglioma

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