Argininosuccinate synthase


NameArgininosuccinate synthase
Synonyms6.3.4.5 ASS Citrulline--aspartate ligase
Gene NameASS1
OrganismHuman
Amino acid sequence
>lcl|BSEQ0016170|Argininosuccinate synthase
MSSKGSVVLAYSGGLDTSCILVWLKEQGYDVIAYLANIGQKEDFEEARKKALKLGAKKVF
IEDVSREFVEEFIWPAIQSSALYEDRYLLGTSLARPCIARKQVEIAQREGAKYVSHGATG
KGNDQVRFELSCYSLAPQIKVIAPWRMPEFYNRFKGRNDLMEYAKQHGIPIPVTPKNPWS
MDENLMHISYEAGILENPKNQAPPGLYTKTQDPAKAPNTPDILEIEFKKGVPVKVTNVKD
GTTHQTSLELFMYLNEVAGKHGVGRIDIVENRFIGMKSRGIYETPAGTILYHAHLDIEAF
TMDREVRKIKQGLGLKFAELVYTGFWHSPECEFVRHCIAKSQERVEGKVQVSVLKGQVYI
LGRESPLSLYNEELVSMNVQGDYEPTDATGFININSLRLKEYHRLQSKVTAK
Number of residues412
Molecular Weight46530.055
Theoretical pI8.18
GO Classification
Functions
    argininosuccinate synthase activity
    poly(A) RNA binding
    ATP binding
    toxic substance binding
    identical protein binding
    amino acid binding
Processes
    cellular response to dexamethasone stimulus
    response to drug
    response to mycotoxin
    response to estradiol
    cellular response to glucagon stimulus
    arginine biosynthetic process
    acute-phase response
    urea cycle
    argininosuccinate metabolic process
    kidney development
    midgut development
    cellular response to oleic acid
    cellular nitrogen compound metabolic process
    response to growth hormone
    polyamine metabolic process
    cellular response to cAMP
    small molecule metabolic process
    cellular response to ammonium ion
    cellular response to lipopolysaccharide
    aspartate metabolic process
    cellular response to laminar fluid shear stress
    liver development
    citrulline metabolic process
    response to nutrient
    positive regulation of nitric oxide biosynthetic process
    negative regulation of leukocyte cell-cell adhesion
    response to zinc ion
    cellular response to amine stimulus
    cellular response to amino acid stimulus
    aging
    cellular response to interferon-gamma
    cellular response to tumor necrosis factor
    diaphragm development
Components
    lysosome
    mitochondrial outer membrane
    perikaryon
    cytoplasm
    cytosol
    extracellular exosome
    nucleus
    cell body fiber
    endoplasmic reticulum
General FunctionToxic substance binding
Specific FunctionIs indirectly involved in the control of blood pressure.
Transmembrane Regions
GenBank Protein ID28872
UniProtKB IDP00966
UniProtKB Entry NameASSY_HUMAN
Cellular LocationNone
Gene sequence
>lcl|BSEQ0016171|Argininosuccinate synthase (ASS1)
ATGTCCAGCAAAGGCTCCGTGGTTCTGGCCTACAGTGGCGGCCTGGACACCTCGTGCATC
CTCGTGTGGCTGAAGGAACAAGGCTATGACGTCATTGCCTATCTGGCCAACATTGGCCAG
AAGGAAGACTTCGAGGAAGCCAGGAAGAAGGCACTGAAGCTTGGGGCCAAAAAGGTGTTC
ATTGAGGATGTCAGCAGGGAGTTTGTGGAGGAGTTCATCTGGCCGGCCATCCAGTCCAGC
GCACTGTATGAGGACCGCTACCTCCTGGGCACCTCTCTTGCCAGGCCCTGCATCGCCCGC
AAACAAGTGGAAATCGCCCAGCGGGAGGGGGCCAAGTATGTGTCCCACGGCGCCACAGGA
AAGGGGAACGATCAGGTCCGGTTTGAGCTCAGCTGCTACTCACTGGCCCCCCAGATAAAG
GTCATTGCTCCCTGGAGGATGCCTGAATTCTACAACCGGTTCAAGGGCCGCAATGACCTG
ATGGAGTACGCAAAGCAACACGGGATTCCCATCCCGGTCACTCCCAAGAACCCGTGGAGC
ATGGATGAGAACCTCATGCACATCAGCTACGAGGCTGGAATCCTGGAGAACCCCAAGAAC
CAAGCGCCTCCAGGTCTCTACACGAAGACCCAGGACCCAGCCAAAGCCCCCAACACCCCT
GACATTCTCGAGATCGAGTTCAAAAAAGGGGTCCCTGTGAAGGTGACCAACGTCAAGGAT
GGCACCACCCACCAGACCTCCTTGGAGCTCTTCATGTACCTGAACGAAGTCGCGGGCAAG
CATGGCGTGGGCCGTATTGACATCGTGGAGAACCGCTTCATTGGAATGAAGTCCCGAGGT
ATCTACGAGACCCCAGCAGGCACCATCCTTTACCATGCTCATTTAGACATCGAGGCCTTC
ACCATGGACCGGGAAGTGCGCAAAATCAAACAAGGCCTGGGCTTGAAATTTGCTGAGCTG
GTGTATACCGGTTTCTGGCACAGCCCTGAGTGTGAATTTGTCCGCCACTGCATCGCCAAG
TCCCAGGAGCGAGTGGAAGGGAAAGTGCAGGTGTCCGTCCTCAAGGGCCAGGTGTACATC
CTCGGCCGGGAGTCCCCACTGTCTCTCTACAATGAGGAGCTGGTGAGCATGAACGTGCAG
GGTGATTATGAGCCAACTGATGCCACCGGGTTCATCAACATCAATTCCCTCAGGCTGAAG
GAATATCATCGTCTCCAGAGCAAGGTCACTGCCAAATAG
GenBank Gene IDX01630
GeneCard IDNone
GenAtlas IDASS1
HGNC IDHGNC:758
Chromosome Location9
Locus9q34.1
References
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  2. Kobayashi K, Rosenbloom C, Beaudet AL, O'Brien WE: Additional mutations in argininosuccinate synthetase causing citrullinemia. Mol Biol Med. 1991 Feb;8(1):95-100.[1943692 ]
  3. Kobayashi K, Jackson MJ, Tick DB, O'Brien WE, Beaudet AL: Heterogeneity of mutations in argininosuccinate synthetase causing human citrullinemia. J Biol Chem. 1990 Jul 5;265(19):11361-7.[2358466 ]
  4. Karlberg T, Collins R, van den Berg S, Flores A, Hammarstrom M, Hogbom M, Holmberg Schiavone L, Uppenberg J: Structure of human argininosuccinate synthetase. Acta Crystallogr D Biol Crystallogr. 2008 Mar;64(Pt 3):279-86. doi: 10.1107/S0907444907067455. Epub 2008 Feb 20.[18323623 ]
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Related FRC


FRCD ID Name Exact Mass Structure



L-Arginine




174.204



Citrulline




175.188



Fumonisin B1




721.838