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Identification
HMDB Protein ID HMDBP00249
Secondary Accession Numbers
  • 5481
  • HMDBP03836
Name Succinate-semialdehyde dehydrogenase, mitochondrial
Synonyms
  1. Aldehyde dehydrogenase family 5 member A1
  2. NAD(+)-dependent succinic semialdehyde dehydrogenase
Gene Name ALDH5A1
Protein Type Unknown
Biological Properties
General Function Involved in oxidoreductase activity
Specific Function Catalyzes one step in the degradation of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA).
Pathways
  • 2-Hydroxyglutric Aciduria (D And L Form)
  • 4-aminobutanoate degradation
  • 4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase Deficiency
  • Alanine, aspartate and glutamate metabolism
  • Butanoate metabolism
  • Glutamate Metabolism
  • Homocarnosinosis
  • Hyperinsulinism-Hyperammonemia Syndrome
  • Succinic semialdehyde dehydrogenase deficiency
Reactions
Succinic acid semialdehyde + NAD + Water → Succinic acid + NADH details
Succinic acid semialdehyde + NAD + Water → Succinic acid + NADH + Hydrogen Ion details
GO Classification
Biological Process
glutamine metabolic process
gamma-aminobutyric acid catabolic process
acetate metabolic process
galactosylceramide metabolic process
glucosylceramide metabolic process
glycerophospholipid metabolic process
glutathione metabolic process
glutamate metabolic process
glucose metabolic process
neurotransmitter catabolic process
neurotransmitter secretion
protein homotetramerization
succinate metabolic process
respiratory electron transport chain
post-embryonic development
central nervous system development
Cellular Component
mitochondrial matrix
mitochondrion
Function
catalytic activity
oxidoreductase activity
oxidoreductase activity, acting on the aldehyde or oxo group of donors
oxidoreductase activity, acting on the aldehyde or oxo group of donors, nad or nadp as acceptor
Molecular Function
NAD binding
succinate-semialdehyde dehydrogenase (NAD+) activity
succinate-semialdehyde dehydrogenase [NAD(P)+] activity
carboxylic acid binding
protein homodimerization activity
Process
metabolic process
oxidation reduction
Cellular Location
  1. Mitochondrion
Gene Properties
Chromosome Location 6
Locus 6p22
SNPs ALDH5A1
Gene Sequence
>1608 bp
ATGGCGACCTGCATTTGGCTGCGGAGCTGTGGGGCCCGGCGCCTCGGGTCGACGTTTCCA
GGCTGCCGCCTCCGCCCCCGCGCCGGCGGCCTGGTCCCTGCCTCCGGGCCTGCGCCCGGC
CCGGCCCAGCTCCGCTGCTACGCTGGGCGCCTGGCGGGCCTCTCTGCGGCGCTGCTGCGC
ACCGACAGCTTCGTGGGCGGCCGCTGGCTCCCGGCCGCCGCCACCTTCCCCGTGCAAGAC
CCGGCCAGCGGCGCCGCTCTGGGCATGGTAGCCGACTGCGGGGTGCGAGAGGCCCGCGCC
GCCGTGCGCGCTGCCTACGAGGCTTTCTGCCGCTGGAGGGAGGTCTCCGCCAAGGAGAGG
AGTTCATTACTTCGGAAGTGGTACAATTTAATGATACAAAATAAGGATGACCTTGCCAGA
ATAATCACAGCTGAAAGTGGAAAGCCACTGAAGGAGGCACATGGAGAAATTCTCTATTCC
GCCTTTTTCCTAGAGTGGTTCTCTGAGGAAGCCCGCCGTGTTTACGGAGACATTATCCAC
ACCCCGGCAAAGGACAGGCGGGCCCTGGTCCTCAAGCAGCCCATAGGCGTGGCTGCAGTC
ATCACCCCGTGGAATTTCCCCAGTGCCATGATCACCCGGAAGGTGGGGGCCGCCCTGGCA
GCCGGCTGTACTGTCGTGGTGAAGCCTGCCGAAGACACGCCCTTCTCCGCCCTGGCCCTG
GCTGAGCTTGCAAGCCAGGCTGGGATTCCTTCAGGTGTATACAATGTTATTCCCTGTTCT
CGAAAGAATGCCAAGGAAGTAGGGGAGGCAATTTGTACTGATCCTCTGGTGTCCAAAATT
TCCTTTACTGGTTCAACAACTACAGGAAAGATCCTGTTGCACCACGCAGCAAACTCTGTG
AAAAGGGTCTCTATGGAGCTGGGCGGCCTTGCTCCATTTATAGTATTTGACAGTGCCAAC
GTGGACCAGGCTGTAGCAGGGGCCATGGCATCTAAATTTAGGAACACTGGACAGACTTGT
GTTTGCTCAAACCAATTCTTGGTGCAAAGGGGCATCCATGATGCCTTTGTAAAAGCATTC
GCCGAGGCCATGAAGAAGAACCTGCGCGTAGGTAATGGATTTGAGGAAGGAACTACTCAG
GGCCCATTAATTAATGAAAAAGCGGTAGAAAAGGTGGAGAAACAGGTGAATGATGCCGTT
TCTAAAGGTGCCACCGTTGTGACAGGTGGAAAACGACACCAACTTGGAAAAAATTTCTTT
GAGCCTACCCTGCTGTGCAATGTCACCCAGGACATGCTGTGCACTCATGAAGAGACTTTC
GGGCCTCTGGCACCAGTTATCAAGTTCGATACAGAGGAGGAGGCTATAGCAATCGCTAAC
GCAGCTGATGTTGGGTTAGCAGGTTATTTTTACTCTCAAGACCCAGCCCAGATCTGGAGA
GTGGCAGAGCAGCTGGAAGTGGGCATGGTTGGCGTCAACGAAGGATTAATTTCCTCTGTG
GAGTGCCCTTTTGGTGGAGTGAAGCAGTCCGGCCTTGGGCGAGAGGGGTCCAAGTATGGC
ATTGATGAGTATCTGGAACTCAAGTATGTGTGTTACGGGGGCTTGTAG
Protein Properties
Number of Residues 535
Molecular Weight 57214.23
Theoretical pI 8.287
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Succinate-semialdehyde dehydrogenase, mitochondrial
MATCIWLRSCGARRLGSTFPGCRLRPRAGGLVPASGPAPGPAQLRCYAGRLAGLSAALLR
TDSFVGGRWLPAAATFPVQDPASGAALGMVADCGVREARAAVRAAYEAFCRWREVSAKER
SSLLRKWYNLMIQNKDDLARIITAESGKPLKEAHGEILYSAFFLEWFSEEARRVYGDIIH
TPAKDRRALVLKQPIGVAAVITPWNFPSAMITRKVGAALAAGCTVVVKPAEDTPFSALAL
AELASQAGIPSGVYNVIPCSRKNAKEVGEAICTDPLVSKISFTGSTTTGKILLHHAANSV
KRVSMELGGLAPFIVFDSANVDQAVAGAMASKFRNTGQTCVCSNQFLVQRGIHDAFVKAF
AEAMKKNLRVGNGFEEGTTQGPLINEKAVEKVEKQVNDAVSKGATVVTGGKRHQLGKNFF
EPTLLCNVTQDMLCTHEETFGPLAPVIKFDTEEEAIAIANAADVGLAGYFYSQDPAQIWR
VAEQLEVGMVGVNEGLISSVECPFGGVKQSGLGREGSKYGIDEYLELKYVCYGGL
GenBank ID Protein 3766467
UniProtKB/Swiss-Prot ID P51649
UniProtKB/Swiss-Prot Entry Name SSDH_HUMAN
PDB IDs
GenBank Gene ID Y11192
GeneCard ID ALDH5A1
GenAtlas ID ALDH5A1
HGNC ID HGNC:408
References
General References
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