The AASS gene provides instructions for making an enzyme called alpha-aminoadipic semialdehyde synthase. This enzyme is found in many tissues, including the liver.
Alpha-aminoadipic semialdehyde synthase is involved in the breakdown of lysine, which is a building block (amino acid) of many different proteins. Amino acids are broken down when they are no longer needed. Alpha-aminoadipic semialdehyde synthase is called a bifunctional enzyme because it performs two functions during the breakdown of lysine. First, it converts lysine to a molecule called saccharopine, and then it converts saccharopine to a molecule called alpha-aminoadipic semialdehyde.
Other Names
alpha-aminoadipate semialdehyde synthase; LKR/SDH; LKRSDH; LORSDH; lysine-ketoglutarate reductase /saccharopine dehydrogenase; saccharopine dehydrogenase (NAD(+), L-glutamate-forming); saccharopine dehydrogenase (NADP(+), L-lysine-forming)
Related Genetics Topics
Identifiers
- NCBI Gene: 10157
- OMIM: 605113
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
