Entity Details

Primary name ASSY_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionP00966
EntryNameASSY_HUMAN
FullNameArgininosuccinate synthase
TaxID9606
Evidenceevidence at protein level
Length412
SequenceStatuscomplete
DateCreated1986-07-21
DateModified2021-06-02

Ontological Relatives

GenesASS1

GO terms

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GOName
GO:0000050 urea cycle
GO:0000052 citrulline metabolic process
GO:0000053 argininosuccinate metabolic process
GO:0001822 kidney development
GO:0001889 liver development
GO:0003723 RNA binding
GO:0004055 argininosuccinate synthase activity
GO:0005524 ATP binding
GO:0005634 nucleus
GO:0005737 cytoplasm
GO:0005741 mitochondrial outer membrane
GO:0005764 lysosome
GO:0005783 endoplasmic reticulum
GO:0005829 cytosol
GO:0006526 arginine biosynthetic process
GO:0006531 aspartate metabolic process
GO:0006953 acute-phase response
GO:0007494 midgut development
GO:0007568 aging
GO:0007584 response to nutrient
GO:0007623 circadian rhythm
GO:0010043 response to zinc ion
GO:0010046 response to mycotoxin
GO:0015643 toxic substance binding
GO:0016597 amino acid binding
GO:0032355 response to estradiol
GO:0042493 response to drug
GO:0042802 identical protein binding
GO:0043204 perikaryon
GO:0045429 positive regulation of nitric oxide biosynthetic process
GO:0060416 response to growth hormone
GO:0060539 diaphragm development
GO:0070062 extracellular exosome
GO:0070852 cell body fiber
GO:0071222 cellular response to lipopolysaccharide
GO:0071230 cellular response to amino acid stimulus
GO:0071242 cellular response to ammonium ion
GO:0071320 cellular response to cAMP
GO:0071346 cellular response to interferon-gamma
GO:0071356 cellular response to tumor necrosis factor
GO:0071377 cellular response to glucagon stimulus
GO:0071400 cellular response to oleic acid
GO:0071418 cellular response to amine stimulus
GO:0071499 cellular response to laminar fluid shear stress
GO:0071549 cellular response to dexamethasone stimulus
GO:1903038 negative regulation of leukocyte cell-cell adhesion

Subcellular Location

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Subcellular Location
Cytoplasm

Domains

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DomainNameCategoryType
IPR001518 Argininosuccinate synthaseFamilyFamily
IPR014729 Rossmann-like alpha/beta/alpha sandwich foldFamilyHomologous superfamily
IPR018223 Argininosuccinate synthase, conserved siteSiteConserved site
IPR023434 Argininosuccinate synthase, type 1 subfamilyFamilyFamily
IPR024074 Argininosuccinate synthetase, catalytic/multimerisation domain bodyFamilyHomologous superfamily

Diseases

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Disease IDSourceNameDescription
215700 OMIMCitrullinemia 1 (CTLN1)The classic form of citrullinemia, an autosomal recessive disease characterized primarily by elevated serum and urine citrulline levels. Ammonia intoxication is another manifestation. It is a disorder of the urea cycle, usually manifesting in the first few days of life. Affected infants appear normal at birth, but as ammonia builds up in the body they present symptoms such as lethargy, poor feeding, vomiting, seizures and loss of consciousness. Less commonly, a milder form can develop later in childhood or adulthood. The disease is caused by variants affecting the gene represented in this entry.

Drugs

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DrugNameSourceType
DB00125 ArginineDrugbanksmall molecule
DB00128 Aspartic acidDrugbanksmall molecule
DB00155 CitrullineDrugbanksmall molecule
DB00171 ATPDrugbanksmall molecule