ⓘ Alpha-aminoadipic semialdehyde synthase, mitochondrial

                                     

ⓘ Alpha-aminoadipic semialdehyde synthase, mitochondrial

Alpha-aminoadipic semialdehyde synthase is an enzyme encoded by the AASS gene in humans and is involved in their major lysine degradation pathway. It is similar to the separate enzymes coded for by the LYS1 and LYS9 genes in yeast, and related to, although not similar in structure, the bifunctional enzyme found in plants. In humans, mutations in the AASS gene, and the corresponding alpha-aminoadipic semialdehyde synthase enzyme are associated with familial hyperlysinemia. This condition is inherited in an autosomal recessive pattern and is not considered a particularly negative condition, thus making it a rare disease.

                                     
  • AASS or Aass may refer to: AASS gene Alpha - aminoadipic semialdehyde synthase mitochondrial American Anti - Slavery Society AASS abolitionist society
  • Gould SJ, Geraghty MT June 2000 Identification of the alpha - aminoadipic semialdehyde synthase gene, which is defective in familial hyperlysinemia American
  • AGK: encoding enzyme mitochondrial acylglycerol kinase AASS: encoding enzyme Alpha - aminoadipic semialdehyde synthase mitochondrial ARHGEF35: encoding protein
  • where fatty acid synthase resides. The acetyl - CoA is transported as a citrate, which has been previously formed in the mitochondrial matrix from acetyl - coA