Names & Taxonomy
- Uniprot ID:
- Q9UDR5
- Entry Name:
- AASS_HUMAN
- Status:
- reviewed
- Protein Names:
- Alpha-aminoadipic semialdehyde synthase, mitochondrial (LKR/SDH) [Includes: Lysine ketoglutarate reductase (LKR) (LOR) (EC 1.5.1.8); Saccharopine dehydrogenase (SDH) (EC 1.5.1.9)]
- Gene Names:
- AASS
- Gene Names Primary:
- AASS
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 926
- Sequence:
- MLQVHRTGLGRLGVSLSKGLHHKAVLAVRREDVNAWERRAPLAPKHIKGITNLGYKVLIQPSNRRAIHDKDYVKAGGILQEDISEACLILGVKRPPEEKLMSRKTYAFFSHTIKAQEANMGLLDEILKQEIRLIDYEKMVDHRGVRVVAFGQWAGVAGMINILHGMGLRLLALGHHTPFMHIGMAHNYRNSSQAVQAVRDAGYEISLGLMPKSIGPLTFVFTGTGNVSKGAQAIFNELPCEYVEPHELKEVSQTGDLRKVYGTVLSRHHHLVRKTDAVYDPAEYDKHPERYISRFNTDIAPYTTCLINGIYWEQNTPRLLTRQDAQSLLAPGKFSPAGVEGCPALPHKLVAICDISADTGGSIEFMTECTTIEHPFCMYDADQHIIHDSVEGSGILMCSIDNLPAQLPIEATECFGDMLYPYVEEMILSDATQPLESQNFSPVVRDAVITSNGTLPDKYKYIQTLRESRERAQSLSMGTRRKVLVLGSGYISEPVLEYLSRDGNIEITVGSDMKNQIEQLGKKYNINPVSMDICKQEEKLGFLVAKQDLVISLLPYVLHPLVAKACITNKVNMVTASYITPALKELEKSVEDAGITIIGELGLDPGLDHMLAMETIDKAKEVGATIESYISYCGGLPAPEHSNNPLRYKFSWSPVGVLMNVMQSATYLLDGKVVNVAGGISFLDAVTSMDFFPGLNLEGYPNRDSTKYAEIYGISSAHTLLRGTLRYKGYMKALNGFVKLGLINREALPAFRPEANPLTWKQLLCDLVGISPSSEHDVLKEAVLKKLGGDNTQLEAAEWLGLLGDEQVPQAESILDALSKHLVMKLSYGPEEKDMIVMRDSFGIRHPSGHLEHKTIDLVAYGDINGFSAMAKTVGLPTAMAAKMLLDGEIGAKGLMGPFSKEIYGPILERIKAEGIIYTTQSTIKP
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion
Function
- Function:
- Bifunctional enzyme that catalyzes the first two steps in lysine degradation. The N-terminal and the C-terminal contain lysine-ketoglutarate reductase and saccharopine dehydrogenase activity, respectively.
- Pathway:
- Amino-acid degradation; L-lysine degradation via saccharopine pathway; glutaryl-CoA from L-lysine: step 1/6.; Amino-acid degradation; L-lysine degradation via saccharopine pathway; glutaryl-CoA from L-lysine: step 2/6.
- Catalytic Activity:
- N(6)-(L-1,3-dicarboxypropyl)-L-lysine + NADP(+) + H(2)O = L-lysine + 2-oxoglutarate + NADPH.; N(6)-(L-1,3-dicarboxypropyl)-L-lysine + NAD(+) + H(2)O = L-glutamate + (S)-2-amino-6-oxohexanoate + NADH.
- Gene Ontology Go:
- intracellular membrane-bounded organelle
mitochondrial matrix
mitochondrion
saccharopine dehydrogenase (NAD+, L-glutamate-forming) activity
saccharopine dehydrogenase (NADP+, L-lysine-forming) activity
cellular nitrogen compound metabolic process
L-lysine catabolic process to acetyl-CoA via saccharopine
lysine catabolic process
protein tetramerization
small molecule metabolic process - Gene Ontology Biological Process:
- cellular nitrogen compound metabolic process
L-lysine catabolic process to acetyl-CoA via saccharopine
lysine catabolic process
protein tetramerization
small molecule metabolic process - Gene Ontology Molecular Function:
- saccharopine dehydrogenase (NAD+, L-glutamate-forming) activity
saccharopine dehydrogenase (NADP+, L-lysine-forming) activity - Gene Ontology Cellular Component:
- intracellular membrane-bounded organelle
mitochondrial matrix
mitochondrion - Keywords:
- Acetylation
Complete proteome
Mitochondrion
Multifunctional enzyme
NAD
NADP
Oxidoreductase
Reference proteome
Transit peptide