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Identification
HMDB Protein ID HMDBP00293
Secondary Accession Numbers
  • 5528
  • HMDBP03799
Name Alpha-aminoadipic semialdehyde dehydrogenase
Synonyms
  1. Aldehyde dehydrogenase family 7 member A1
  2. Alpha-AASA dehydrogenase
  3. Antiquitin-1
  4. Betaine aldehyde dehydrogenase
  5. Delta1-piperideine-6-carboxylate dehydrogenase
  6. P6c dehydrogenase
Gene Name ALDH7A1
Protein Type Unknown
Biological Properties
General Function Involved in oxidoreductase activity
Specific Function Multifunctional enzyme mediating important protective effects. Metabolizes betaine aldehyde to betaine, an important cellular osmolyte and methyl donor. Protects cells from oxidative stress by metabolizing a number of lipid peroxidation-derived aldehydes. Involved in lysine catabolism.
Pathways
  • 2-aminoadipic 2-oxoadipic aciduria
  • Arginine and proline metabolism
  • Ascorbate and aldarate metabolism
  • beta-Alanine metabolism
  • betaine biosynthesis via choline pathway
  • Betaine Metabolism
  • fatty acid metabolism
  • Glutaric Aciduria Type I
  • Glycerolipid metabolism
  • Glycine, serine and threonine metabolism
  • Glycolysis / Gluconeogenesis
  • Histidine metabolism
  • Hyperlysinemia I, Familial
  • Hyperlysinemia II or Saccharopinuria
  • Lysine biosynthesis
  • Lysine Degradation
  • Lysine degradation
  • Propanoate metabolism
  • Pyridoxine dependency with seizures
  • Pyruvate metabolism
  • Saccharopinuria/Hyperlysinemia II
  • Sarcosine Oncometabolite Pathway
  • Tryptophan metabolism
  • Valine, leucine and isoleucine degradation
Reactions
(S)-2-amino-6-oxohexanoate + NAD(P)(+) + Water → Aminoadipic acid + NAD(P)H details
Betaine aldehyde + NAD + Water → Trimethylammonioacetate + NADH details
An aldehyde + NAD + Water → a carboxylate + NADH details
Aldehyde + NAD + Water → Fatty acid + NADH + Hydrogen Ion details
Acetaldehyde + NAD + Water → Acetic acid + NADH + Hydrogen Ion details
3-Aminopropionaldehyde + NAD + Water → beta-Alanine + NADH + Hydrogen Ion details
Glyceraldehyde + NAD + Water → Glyceric acid + NADH + Hydrogen Ion details
4-Aminobutyraldehyde + NADP + Water → gamma-Aminobutyric acid + NADPH + Hydrogen Ion details
4-Aminobutyraldehyde + NAD + Water → gamma-Aminobutyric acid + NADH + Hydrogen Ion details
Betaine aldehyde + NAD + Water → Trimethylammonioacetate + NADH + Hydrogen Ion details
Betaine aldehyde + NADP + Water → Trimethylammonioacetate + NADPH + Hydrogen Ion details
Indoleacetaldehyde + NAD + Water → Indoleacetic acid + NADH + Hydrogen Ion details
2-Propyn-1-al + NAD + Water → Propynoic acid + NADH + Hydrogen Ion details
D-Glucurono-6,3-lactone + NAD + Water → Glucaric acid + NADH + Hydrogen Ion details
Aminoadipic acid + Adenosine triphosphate → L-2-Aminoadipate adenylate + Pyrophosphate details
(S)-2-amino-6-oxohexanoate + NAD + Water → Aminoadipic acid + NADH + Hydrogen Ion details
(S)-2-amino-6-oxohexanoate + NADP + Water → Aminoadipic acid + NADPH + Hydrogen Ion details
4-Trimethylammoniobutanal + NAD + Water → 4-Trimethylammoniobutanoic acid + NADH + Hydrogen Ion details
(S)-Methylmalonic acid semialdehyde + NAD + Water → Methylmalonic acid + NADH + Hydrogen Ion details
Imidazole-4-acetaldehyde + NAD + Water → Imidazoleacetic acid + NADH + Hydrogen Ion details
alpha-Aminoadipoyl-S-acyl enzyme + NADPH + Hydrogen Ion → (S)-2-amino-6-oxohexanoate + Holo-Lys2 + NADP details
3a,7a-Dihydroxy-5b-cholestan-26-al + NAD + Water → 3a,7a-Dihydroxy-5b-cholestanate + NADH + Hydrogen Ion details
L-2-Aminoadipate adenylate + Holo-Lys2 → alpha-Aminoadipoyl-S-acyl enzyme + Adenosine monophosphate details
5-Hydroxyindoleacetaldehyde + NAD + Water → 5-Hydroxyindoleacetic acid + Hydrogen Ion + NADH details
N4-Acetylaminobutanal + NAD + Water → 4-Acetamidobutanoic acid + NADH + Hydrogen Ion details
GO Classification
Biological Process
sensory perception of sound
cellular nitrogen compound metabolic process
cellular aldehyde metabolic process
lysine catabolic process
glycine betaine biosynthetic process from choline
Cellular Component
cytosol
mitochondrial matrix
nucleus
Function
catalytic activity
oxidoreductase activity
Molecular Function
aldehyde dehydrogenase (NAD) activity
betaine-aldehyde dehydrogenase activity
L-aminoadipate-semialdehyde dehydrogenase activity
Process
metabolic process
oxidation reduction
Cellular Location
  1. Isoform 2:Cytoplasm
  2. cytosol
Gene Properties
Chromosome Location 5
Locus 5q31
SNPs ALDH7A1
Gene Sequence
>1620 bp
ATGTGGCGCCTTCCTCGCGCGCTGTGTGTGCACGCTGCAAAGACCAGCAAGCTCTCTGGA
CCTTGGAGCAGGCCTGCCGCCTTCATGTCCACTCTCCTCATCAATCAGCCCCAGTATGCG
TGGCTGAAAGAGCTGGGGCTCCGCGAGGAAAACGAGGGCGTGTATAATGGAAGCTGGGGA
GGCCGGGGAGAGGTTATTACGACCTATTGCCCTGCTAACAACGAGCCAATAGCAAGAGTC
CGACAGGCCAGTGTGGCAGACTATGAAGAAACTGTAAAGAAAGCAAGAGAAGCATGGAAA
ATCTGGGCAGATATTCCTGCTCCAAAACGAGGAGAAATAGTAAGACAGATTGGCGATGCC
TTGCGGGAGAAGATCCAAGTACTAGGAAGCTTGGTGTCTTTGGAGATGGGGAAAATCTTA
GTGGAAGGTGTGGGTGAAGTTCAGGAGTATGTGGATATCTGTGACTATGCTGTTGGTTTA
TCAAGGATGATTGGAGGACCTATCTTGCCTTCTGAAAGATCTGGCCATGCACTGATTGAG
CAGTGGAATCCCGTAGGCCTGGTTGGAATCATCACGGCATTCAATTTCCCTGTGGCAGTG
TATGGTTGGAACAACGCCATCGCCATGATCTGTGGAAATGTCTGCCTCTGGAAAGGAGCT
CCAACCACTTCCCTCATTAGTGTGGCTGTCACAAAGATAATAGCCAAGGTTCTGGAGGAC
AACAAGCTGCCTGGTGCAATTTGTTCCTTGACTTGTGGTGGAGCAGATATTGGCACAGCA
ATGGCCAAAGATGAACGAGTGAACCTGCTGTCCTTCACTGGGAGCACTCAGGTGGGAAAA
CAGGTGGGCCTGATGGTGCAGGAGAGGTTTGGGAGAAGTCTGTTGGAACTTGGAGGAAAC
AATGCCATTATTGCCTTTGAAGATGCAGACCTCAGCTTAGTTGTTCCATCAGCTCTCTTC
GCTGCTGTGGGAACAGCTGGCCAGAGGTGTACCACTGCGAGGCGACTGTTTATACATGAA
AGCATCCATGATGAGGTTGTAAACAGACTTAAAAAGGCCTATGCACAGATCCGAGTTGGG
AACCCATGGGACCCTAATGTTCTCTATGGGCCACTCCACACCAAGCAGGCAGTGAGCATG
TTTCTTGGAGCAGTGGAAGAAGCAAAGAAAGAAGGTGGCACAGTGGTCTATGGGGGCAAG
GTTATGGATCGCCCTGGAAATTATGTAGAACCGACAATTGTGACAGGTCTTGGCCACGAT
GCGTCCATTGCACACACAGAGACTTTTGCTCCGATTCTCTATGTCTTTAAATTCAAGAAT
GAAGAAGAGGTCTTTGCATGGAATAATGAAGTAAAACAGGGACTTTCAAGTAGCATCTTT
ACCAAAGATCTGGGCAGAATCTTTCGCTGGCTTGGACCTAAAGGATCAGACTGTGGCATT
GTAAATGTCAACATTCCAACAAGTGGGGCTGAGATTGGAGGTGCCTTTGGAGGAGAAAAG
CACACTGGTGGTGGCAGGGAGTCTGGCAGTGATGCCTGGAAACAGTACATGAGAAGGTCT
ACTTGTACTATCAACTACAGTAAAGACCTTCCTCTGGCCCAAGGAATCAAGTTTCAGTAA
Protein Properties
Number of Residues 539
Molecular Weight 58486.74
Theoretical pI 7.987
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Alpha-aminoadipic semialdehyde dehydrogenase
MWRLPRALCVHAAKTSKLSGPWSRPAAFMSTLLINQPQYAWLKELGLREENEGVYNGSWG
GRGEVITTYCPANNEPIARVRQASVADYEETVKKAREAWKIWADIPAPKRGEIVRQIGDA
LREKIQVLGSLVSLEMGKILVEGVGEVQEYVDICDYAVGLSRMIGGPILPSERSGHALIE
QWNPVGLVGIITAFNFPVAVYGWNNAIAMICGNVCLWKGAPTTSLISVAVTKIIAKVLED
NKLPGAICSLTCGGADIGTAMAKDERVNLLSFTGSTQVGKQVGLMVQERFGRSLLELGGN
NAIIAFEDADLSLVVPSALFAAVGTAGQRCTTARRLFIHESIHDEVVNRLKKAYAQIRVG
NPWDPNVLYGPLHTKQAVSMFLGAVEEAKKEGGTVVYGGKVMDRPGNYVEPTIVTGLGHD
ASIAHTETFAPILYVFKFKNEEEVFAWNNEVKQGLSSSIFTKDLGRIFRWLGPKGSDCGI
VNVNIPTSGAEIGGAFGGEKHTGGGRESGSDAWKQYMRRSTCTINYSKDLPLAQGIKFQ
GenBank ID Protein 188035924
UniProtKB/Swiss-Prot ID P49419
UniProtKB/Swiss-Prot Entry Name AL7A1_HUMAN
PDB IDs
GenBank Gene ID NM_001182.3
GeneCard ID ALDH7A1
GenAtlas ID ALDH7A1
HGNC ID HGNC:877
References
General References
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