Identification
BMDB Protein ID BMDBP00631
Secondary Accession Numbers None
Name Alpha-aminoadipic semialdehyde synthase, mitochondrial
Synonyms Not Available
Gene Name AASS
Protein Type Enzyme
Biological Properties
General Function Amino acid transport and metabolism
Specific Function Bifunctional enzyme that catalyzes the first two steps in lysine degradation. The N-terminal and the C-terminal contain lysine-oxoglutarate reductase and saccharopine dehydrogenase activity, respectively (By similarity).
Pathways
  • Amino-acid degradation
  • Glutaryl-CoA from L-lysine: step 1/6.
  • Glutaryl-CoA from L-lysine: step 2/6
  • L-lysine degradation via saccharopine pathway
  • Lysine Degradation
Reactions
L-Lysine + NADPH + Oxoglutaric acid → Saccharopine + NADP + Water details
Saccharopine + Water + NAD → Allysine + L-Glutamic acid + NADH details
GO Classification Not Available
Cellular Location Not Available
Gene Properties
Chromosome Location 4
Locus Not Available
SNPs AASS
Gene Sequence
>2781 bp
ATGCTGCGAGTGTCCAGAACCAAACTGGGCAGGCTGAGCCCCAGCCTCTCCAGAGGGCTT
CACCACAAGGCGGTGATGGCCTTGAGGCGGGAGGATGTGAATGCCTGGGAAAGGCGGGCT
CCTCTGGCTCCCCGGCACGTCAAAGGGATCACCAACCTGGGATACAAGGTCTTAATACAG
CCTTCCAATCGGCGAGCCATTCACGATAAGGAATATGTCAAAGCTGGTGGCATTCTCCAA
GAGGATATTTCTGAAGCTTGTCTAATTTTGGGAGTTAAAAGACCGCCAGAGGAAAAATTA
ATGCCCAAGAAGACTTATGCATTCTTCTCCCACACAATAAAGGCTCAGGAGGCCAATATG
GGTTTGCTGGATGAGATCCTAAAACAGGAAATCCGACTTATTGATTATGAGAAAATGGTG
GATCATAGAGGAATACGGGTGGTGGCATTCGGACAGTGGGCAGGTGTGGCAGGAATAATC
AACATTCTTCATGGGATGGGTTTAAGGCTCCTTGCTTTGGGACATCACACACCTTTTATG
CACATTGGCATGGCTCATAACTACAGGAACAGTGGTCAGGCTGTGCAAGCTGTCCGCGAT
GCTGGCTATGAAATATCTTTGGGTTTGATGCCGAAGTCAATAGGGCCCTTAACATTTGTG
TTCACAGGGACTGGTAATGTATCTAAGGGAGCCCAAGAAATCTTCAATGAATTACCTTGT
GAGTATGTGGAGCCACATGAATTAAAAGAAGTTTCCCAAAATGGAGACCTGAGAAAAGTG
TACGGGACAGTGTTAAGTCGCCATCATCATCTTGTCAGGAAAACAGATGGTGTATATGAC
CCTGTAGAGTATGACAAATACCCCGAGCGCTACATTAGCCGTTTTAATACTGATATTGCA
CCCTATACAACTTGTTTAATTAACGGGATCTACTGGGAACAGAACACCCCTCGTCTACTC
ACCCGCCAAGATGCTCAGAGTCTCCTGGCTCCAGGCAAGTCCCCAGTTGCTGGTGTTGAA
GGCTGCCCTGCATTGCCACACAAACTTGTGGCAATATGTGACATTTCAGCGGACACAGGA
GGATCCATTGAGTTTATGACTGAGTGTACGACCATAGAGCGTCCCTTTTGCATGTATGAT
GCAGATCAGCATATTATTCATGACAGTGTGGAAGGCTCTGGGATTCTGATGTGTTCCATT
GACAATTTGCCAGCACAGCTTCCAATTGAATCTACAGAATACTTTGGAGACATGCTTTAC
CCTTATGTTGAAGAAATGATACTATCAGATGCGACACAGCCTCTTGAAAGTCAGAATTTT
TCTCCTGTGGTGCGAGATGCAGTGATTGCATCTAATGGCATGCTGTCTAACAAGTATAAA
TATATCCAGAAACTCCGAGAGAACAGGGAACATGCTCAGTCACTTTCAATGGGCACCAAG
AAAAAGGTCTTGGTTCTTGGATCTGGTTACGTTTCTGAGCCTGTATTGGAATACCTGTTG
AGAGATGACAGTATTGAAATAACAGTAGGTTCTGACATGAAGAATCAAATTGAACAATTA
GGCAAGAAATATAATATTAATCCTGTTAGCCTGCACGTTGGTAAACAAGAAGAGAAGTTG
AGCTCCTTGGTGGCAACACAGGATCTTGTCATCAGCTTATTGCCTTATGTATTGCATCCT
CTTGTGGCCAAGGCATGCATCGCAAGCAAAGTTAACATGATCACTGCAAGCTACATCACC
CCAGCGCTAAAAGAATTAGAAAAGAGTGTGGAAGATGCTGGCATCACAGTCATTGGTGAA
TTGGGATTGGACCCTGGTCTTGATCATATGTTGGCAATGGAAACAATAGATAAGGCCAAA
GAAGTGGGAGCCACGATTGAATCTTATGTTTCCTACTGCGGTGGCCTTCCAGCTCCTGAA
TGTTCAGACAATCCTTTGAGATACAAATTTAGCTGGAGTCCAGTGGGAGTTCTGATGAAC
ATAATGCAGCCCGCTACCTACCTGCTCAACGGAAAGGTTGTGAATGCTGTAGGAGGTGTC
TCCTTTCTGGATTCAGTCACTCCCATGGATTATTTCCCTGGATTAAATTTGGAAGGTTAT
CCTAACAGAGACAGTACGAAGTATGCTGAGATTTATGGCATTCCATCTGCTCACACTTTA
TTACGAGGGACACTGAGGTACAAGGGGTATGCCAAAGCTTTGAGTGGATTTGTGAAGTTG
GGTCTCATAAACAGAGATGCGTTTCCTGCCCTTCAACCTGATGCCAATCCGCTCACCTGG
AAAGAACTCCTCTGTGATCTAGTTGGGATTTCATCCTCTTCCAAATGTGATGTGCTTAAG
GAAGCTGTCTTTAAGAAACTGGGAGGAGACACAACCCAGTTGGAGGCTCTCGAATGGTTA
GGGCTACTCGGGGATGAACAAGTCCCTCAGGCCGAGTCTCTCGTGGATGCCCTCTCCAAG
CATCTGGCCGTGAAACTTTCCTATGGTCCTGGAGAGAAAGATATGATTGTGATGAGAGAC
AGCTTTGGAATCAGACATCCTTCTGGACATTTGGAAAATAAAACTATTGATCTTGTGGTT
TATGGGGATGTCAATGGTTTTTCAGCCATGGCTAAAACTGTGGGGTTACCCACTGCTATG
GCAGCCAAGATGTTACTTGATGGTGAAATTCAAGCTAAAGGCCTGATGGGACCCTTTTCA
AAGGAGATCTATGGACCAATATTGGAGCGAATTAAAGCAGAAGGCATTATGTATACTACA
CAGAGCACAATCAAACTATAA
Protein Properties
Number of Residues 926
Molecular Weight 102084.0
Theoretical pI 6.7
Pfam Domain Function Not Available
Signals
  • None
Transmembrane Regions Not Available
Protein Sequence
>Alpha-aminoadipic semialdehyde synthase, mitochondrial
MLRVSRTKLGRLSPSLSRGLHHKAVMALRREDVNAWERRAPLAPRHVKGITNLGYKVLIQ
PSNRRAIHDKEYVKAGGILQEDISEACLILGVKRPPEEKLMPKKTYAFFSHTIKAQEANM
GLLDEILKQEIRLIDYEKMVDHRGIRVVAFGQWAGVAGIINILHGMGLRLLALGHHTPFM
HIGMAHNYRNSGQAVQAVRDAGYEISLGLMPKSIGPLTFVFTGTGNVSKGAQEIFNELPC
EYVEPHELKEVSQNGDLRKVYGTVLSRHHHLVRKTDGVYDPVEYDKYPERYISRFNTDIA
PYTTCLINGIYWEQNTPRLLTRQDAQSLLAPGKSPVAGVEGCPALPHKLVAICDISADTG
GSIEFMTECTTIERPFCMYDADQHIIHDSVEGSGILMCSIDNLPAQLPIESTEYFGDMLY
PYVEEMILSDATQPLESQNFSPVVRDAVIASNGMLSNKYKYIQKLRENREHAQSLSMGTK
KKVLVLGSGYVSEPVLEYLLRDDSIEITVGSDMKNQIEQLGKKYNINPVSLHVGKQEEKL
SSLVATQDLVISLLPYVLHPLVAKACIASKVNMITASYITPALKELEKSVEDAGITVIGE
LGLDPGLDHMLAMETIDKAKEVGATIESYVSYCGGLPAPECSDNPLRYKFSWSPVGVLMN
IMQPATYLLNGKVVNAVGGVSFLDSVTPMDYFPGLNLEGYPNRDSTKYAEIYGIPSAHTL
LRGTLRYKGYAKALSGFVKLGLINRDAFPALQPDANPLTWKELLCDLVGISSSSKCDVLK
EAVFKKLGGDTTQLEALEWLGLLGDEQVPQAESLVDALSKHLAVKLSYGPGEKDMIVMRD
SFGIRHPSGHLENKTIDLVVYGDVNGFSAMAKTVGLPTAMAAKMLLDGEIQAKGLMGPFS
KEIYGPILERIKAEGIMYTTQSTIKL
GenBank ID Protein AAI53852.1
UniProtKB/Swiss-Prot ID A8E657
UniProtKB/Swiss-Prot Entry Name AASS_BOVIN
PDB IDs Not Available
GenBank Gene ID BC153851
GeneCard ID AASS
GenAtlas ID Not Available
HGNC ID Not Available
References
General References Not Available