ACADSB gene

acyl-CoA dehydrogenase, short/branched chain

The ACADSB gene provides instructions for making an enzyme called 2-methylbutyryl-CoA dehydrogenase, which plays an important role in processing proteins. Normally, the body breaks down proteins from food into smaller parts called amino acids. Amino acids can be further processed to provide energy for the body. In cells throughout the body, 2-methylbutyryl-CoA dehydrogenase is found within specialized structures called mitochondria. Mitochondria convert energy from food to a form that cells can use.

The 2-methylbutyryl-CoA dehydrogenase enzyme helps break down a particular amino acid called isoleucine. Specifically, this enzyme helps with the third step of the process, performing a chemical reaction that converts a molecule called 2-methylbutyryl-CoA to another molecule, tiglyl-CoA. Additional chemical reactions convert tiglyl-CoA into molecules that are used for energy.