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Identification
HMDB Protein ID HMDBP00656
Secondary Accession Numbers
  • 5928
  • HMDBP04157
Name Argininosuccinate synthase
Synonyms
  1. Citrulline--aspartate ligase
Gene Name ASS1
Protein Type Enzyme
Biological Properties
General Function Involved in argininosuccinate synthase activity
Specific Function Not Available
Pathways
  • Alanine, aspartate and glutamate metabolism
  • Arginine and Proline Metabolism
  • Arginine and proline metabolism
  • Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency)
  • Argininemia
  • Argininosuccinic Aciduria
  • Aspartate Metabolism
  • Canavan Disease
  • Carbamoyl Phosphate Synthetase Deficiency
  • Citrullinemia Type I
  • Creatine deficiency, guanidinoacetate methyltransferase deficiency
  • Guanidinoacetate Methyltransferase Deficiency (GAMT Deficiency)
  • Hyperornithinemia with gyrate atrophy (HOGA)
  • Hyperornithinemia-hyperammonemia-homocitrullinuria [HHH-syndrome]
  • Hyperprolinemia Type I
  • Hyperprolinemia Type II
  • Hypoacetylaspartia
  • L-arginine biosynthesis
  • L-arginine:glycine amidinotransferase deficiency
  • Ornithine Aminotransferase Deficiency (OAT Deficiency)
  • Ornithine Transcarbamylase Deficiency (OTC Deficiency)
  • Prolidase Deficiency (PD)
  • Prolinemia Type II
  • urea cycle
  • Urea Cycle
Reactions
Adenosine triphosphate + Citrulline + L-Aspartic acid → Adenosine monophosphate + Pyrophosphate + Argininosuccinic acid details
Adenosine triphosphate + Citrulline + L-Aspartic acid → Adenosine monophosphate + Pyrophosphate + Argininosuccinic acid details
GO Classification
Biological Process
response to zinc ion
acute-phase response
arginine biosynthetic process
argininosuccinate metabolic process
cellular response to amine stimulus
cellular response to amino acid stimulus
cellular response to ammonium ion
cellular response to cAMP
cellular response to oleic acid
cellular response to tumor necrosis factor
diaphragm development
response to growth hormone stimulus
response to mycotoxin
liver development
response to drug
response to nutrient
cellular response to lipopolysaccharide
aging
kidney development
midgut development
response to estradiol stimulus
cellular response to dexamethasone stimulus
urea cycle
cellular response to interferon-gamma
cellular response to glucagon stimulus
Cellular Component
cytosol
endoplasmic reticulum
nucleus
cell body fiber
perikaryon
lysosome
mitochondrial outer membrane
Function
binding
catalytic activity
nucleoside binding
purine nucleoside binding
adenyl nucleotide binding
adenyl ribonucleotide binding
atp binding
ligase activity
argininosuccinate synthase activity
ligase activity, forming carbon-nitrogen bonds
Molecular Function
argininosuccinate synthase activity
toxin binding
ATP binding
Process
arginine biosynthetic process
metabolic process
arginine metabolic process
cellular metabolic process
glutamine family amino acid metabolic process
cellular amino acid and derivative metabolic process
cellular amino acid metabolic process
Cellular Location Not Available
Gene Properties
Chromosome Location 9
Locus 9q34.1
SNPs ASS1
Gene Sequence
>1239 bp
ATGTCCAGCAAAGGCTCCGTGGTTCTGGCCTACAGTGGCGGCCTGGACACCTCGTGCATC
CTCGTGTGGCTGAAGGAACAAGGCTATGACGTCATTGCCTATCTGGCCAACATTGGCCAG
AAGGAAGACTTCGAGGAAGCCAGGAAGAAGGCACTGAAGCTTGGGGCCAAAAAGGTGTTC
ATTGAGGATGTCAGCAGGGAGTTTGTGGAGGAGTTCATCTGGCCGGCCATCCAGTCCAGC
GCACTGTATGAGGACCGCTACCTCCTGGGCACCTCTCTTGCCAGGCCCTGCATCGCCCGC
AAACAAGTGGAAATCGCCCAGCGGGAGGGGGCCAAGTATGTGTCCCACGGCGCCACAGGA
AAGGGGAACGATCAGGTCCGGTTTGAGCTCAGCTGCTACTCACTGGCCCCCCAGATAAAG
GTCATTGCTCCCTGGAGGATGCCTGAATTCTACAACCGGTTCAAGGGCCGCAATGACCTG
ATGGAGTACGCAAAGCAACACGGGATTCCCATCCCGGTCACTCCCAAGAACCCGTGGAGC
ATGGATGAGAACCTCATGCACATCAGCTACGAGGCTGGAATCCTGGAGAACCCCAAGAAC
CAAGCGCCTCCAGGTCTCTACACGAAGACCCAGGACCCAGCCAAAGCCCCCAACACCCCT
GACATTCTCGAGATCGAGTTCAAAAAAGGGGTCCCTGTGAAGGTGACCAACGTCAAGGAT
GGCACCACCCACCAGACCTCCTTGGAGCTCTTCATGTACCTGAACGAAGTCGCGGGCAAG
CATGGCGTGGGCCGTATTGACATCGTGGAGAACCGCTTCATTGGAATGAAGTCCCGAGGT
ATCTACGAGACCCCAGCAGGCACCATCCTTTACCATGCTCATTTAGACATCGAGGCCTTC
ACCATGGACCGGGAAGTGCGCAAAATCAAACAAGGCCTGGGCTTGAAATTTGCTGAGCTG
GTGTATACCGGTTTACGGCCTAGCCCTGAGTGTGAATTTGTCCGCCACTGCATCGCCAAG
TCCCAGGAGCGAGTGGAAGGGAAAGTGCAGGTGTCCGTCCTCAAGGGCCAGGTGTACATC
CTCGGCCGGGAGTCCCCACTGTCTCTCTACAATGAGGAGCTGGTGAGCATGAACGTGCAG
GGTGATTATGAGCCAACTGATGCCACCGGGTTCATCAACATCAATTCCCTCAGGCTGAAG
GAATATCATCGTCTCCAGAGCAAGGTCACTGCCAAATAG
Protein Properties
Number of Residues 412
Molecular Weight 46530.055
Theoretical pI 8.022
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Argininosuccinate synthase
MSSKGSVVLAYSGGLDTSCILVWLKEQGYDVIAYLANIGQKEDFEEARKKALKLGAKKVF
IEDVSREFVEEFIWPAIQSSALYEDRYLLGTSLARPCIARKQVEIAQREGAKYVSHGATG
KGNDQVRFELSCYSLAPQIKVIAPWRMPEFYNRFKGRNDLMEYAKQHGIPIPVTPKNPWS
MDENLMHISYEAGILENPKNQAPPGLYTKTQDPAKAPNTPDILEIEFKKGVPVKVTNVKD
GTTHQTSLELFMYLNEVAGKHGVGRIDIVENRFIGMKSRGIYETPAGTILYHAHLDIEAF
TMDREVRKIKQGLGLKFAELVYTGFWHSPECEFVRHCIAKSQERVEGKVQVSVLKGQVYI
LGRESPLSLYNEELVSMNVQGDYEPTDATGFININSLRLKEYHRLQSKVTAK
GenBank ID Protein 28872
UniProtKB/Swiss-Prot ID P00966
UniProtKB/Swiss-Prot Entry Name ASSY_HUMAN
PDB IDs
GenBank Gene ID X01630
GeneCard ID ASS1
GenAtlas ID ASS1
HGNC ID HGNC:758
References
General References
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