ASS1 One of the enzymes of the urea cycle, the metabolic pathway transforming neurotoxic amonia produced by protein catabolism into inocuous urea in the liver of ureotelic animals. Catalyzes the formation of arginosuccinate from aspartate, citrulline and ATP and together with ASL it is responsible for the biosynthesis of arginine in most body tissues. Belongs to the argininosuccinate synthase family. Type 1 subfamily. Expressed in adult liver. Note: This description may include information from UniProtKB.
Protein type: Amino Acid Metabolism - alanine, aspartate and glutamate; Amino Acid Metabolism - arginine and proline; EC; Endoplasmic reticulum; Ligase; Mitochondrial
Chromosomal Location of human Ortholog: 9q34.11
Cellular Component:  cell body fiber; cytoplasm; cytosol; endoplasmic reticulum; lysosome; mitochondrial outer membrane; nucleoplasm; perikaryon
Molecular Function:  amino acid binding; argininosuccinate synthase activity; ATP binding; identical protein binding; protein binding; toxic substance binding
Biological Process:  acute-phase response; aging; arginine biosynthetic process; argininosuccinate metabolic process; aspartate 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 dexamethasone stimulus; cellular response to glucagon stimulus; cellular response to laminar fluid shear stress; cellular response to lipopolysaccharide; cellular response to oleic acid; cellular response to tumor necrosis factor; cellular response to type II interferon; circadian rhythm; citrulline metabolic process; diaphragm development; kidney development; liver development; midgut development; negative regulation of leukocyte cell-cell adhesion; positive regulation of nitric oxide biosynthetic process; response to estradiol; response to growth hormone; response to mycotoxin; response to nutrient; response to xenobiotic stimulus; response to zinc ion; urea cycle
Disease: Citrullinemia, Classic
Reference #:  P00966 (UniProtKB)
Alt. Names/Synonyms: Argininosuccinate synthase; argininosuccinate synthase 1; argininosuccinate synthetase 1; ASS; ASS1; ASSY; Citrulline--aspartate ligase; citrulline-aspartate ligase; CTLN1; epididymis secretory sperm binding protein
Gene Symbols: ASS1
Molecular weight: 46,530 Da
Basal Isoelectric point: 8.08  Predict pI for various phosphorylation states
Protein-Specific Antibodies, siRNAs or Recombinant Proteins from Cell Signaling Technology® Total Proteins
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Protein Structure Not Found.

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