SDHAF4
Description
The SDHAF4 (succinate dehydrogenase complex assembly factor 4) is a protein-coding gene located on chromosome 6.
SDHAF4 plays a crucial role in the assembly of succinate dehydrogenase (SDH), a key enzyme involved in both the tricarboxylic acid cycle (TCA) and mitochondrial electron transport chain. SDHAF4 binds to the flavoprotein subunit SDHA, specifically when it is bound to FAD, preventing the production of excess reactive oxygen species (ROS). This interaction also facilitates the assembly of SDHA with the iron-sulfur protein subunit SDHB, forming the active catalytic dimer of SDH.
SDHAF4 is also known as C6orf57, Sdh8.
Associated Diseases
- type 2 diabetes mellitus
- very long chain acyl-CoA dehydrogenase deficiency
- Pallister-Hall syndrome
- fragile X syndrome
