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Argininosuccinate Synthetase 1 (ASS1)

CTLN1; Citrulline--aspartate ligase

Argininosuccinate synthetase 1 catalyzes the penultimate step of the arginine biosynthetic pathway. There are approximately 10 to 14 copies of this gene including the pseudogenes scattered across the human genome, among which the one located on chromosome 9 appears to be the only functional gene for argininosuccinate synthetase.

The ASS1 gene encodes ardininosuccinate synthetase-1, a cytosolic urea cycle enzyme mainly expressed in periportal hepatocytes, but also in most other body tissues. The enzyme is a homotetrameric protein composed of 45-kD monomers and is involved in the synthesis of arginine and catalyzes that condensation of citrulline and aspartate to argininosuccinate using ATP.

Organism species: Homo sapiens (Human)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPU528Hu01 Recombinant Argininosuccinate Synthetase 1 (ASS1) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAU528Hu01 Monoclonal Antibody to Argininosuccinate Synthetase 1 (ASS1) WB; IHC; ICC; IP.
PAU528Hu01 Polyclonal Antibody to Argininosuccinate Synthetase 1 (ASS1) WB; IHC; ICC; IF.
Assay Kits CLU528Hu01 CLIA Kit for Argininosuccinate Synthetase 1 (ASS1) CLIA assay for Antigen Detection
ELU528Hu01 ELISA Kit for Argininosuccinate Synthetase 1 (ASS1) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Mus musculus (Mouse)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPU528Mu01 Recombinant Argininosuccinate Synthetase 1 (ASS1) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAU528Mu01 Monoclonal Antibody to Argininosuccinate Synthetase 1 (ASS1) WB; IHC; ICC; IP.
PAU528Mu01 Polyclonal Antibody to Argininosuccinate Synthetase 1 (ASS1) WB; IHC; ICC; IF.
Assay Kits CLU528Mu01 CLIA Kit for Argininosuccinate Synthetase 1 (ASS1) CLIA assay for Antigen Detection
ELU528Mu01 ELISA Kit for Argininosuccinate Synthetase 1 (ASS1) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Rattus norvegicus (Rat)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPU528Ra01 Recombinant Argininosuccinate Synthetase 1 (ASS1) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAU528Ra01 Monoclonal Antibody to Argininosuccinate Synthetase 1 (ASS1) WB; IHC; ICC; IP.
PAU528Ra01 Polyclonal Antibody to Argininosuccinate Synthetase 1 (ASS1) WB; IHC; ICC; IF.
Assay Kits CLU528Ra01 CLIA Kit for Argininosuccinate Synthetase 1 (ASS1) CLIA assay for Antigen Detection
ELU528Ra01 ELISA Kit for Argininosuccinate Synthetase 1 (ASS1) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Canis familiaris; Canine (Dog)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPU528Ca01 Recombinant Argininosuccinate Synthetase 1 (ASS1) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAU528Ca01 Monoclonal Antibody to Argininosuccinate Synthetase 1 (ASS1) WB; IHC; ICC; IP.
PAU528Ca01 Polyclonal Antibody to Argininosuccinate Synthetase 1 (ASS1) WB; IHC; ICC; IF.
Assay Kits CLU528Ca01 CLIA Kit for Argininosuccinate Synthetase 1 (ASS1) CLIA assay for Antigen Detection
ELU528Ca01 ELISA Kit for Argininosuccinate Synthetase 1 (ASS1) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Sus scrofa; Porcine (Pig)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPU528Po01 Recombinant Argininosuccinate Synthetase 1 (ASS1) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAU528Po01 Monoclonal Antibody to Argininosuccinate Synthetase 1 (ASS1) WB; IHC; ICC; IP.
PAU528Po01 Polyclonal Antibody to Argininosuccinate Synthetase 1 (ASS1) WB; IHC; ICC; IF.
Assay Kits CLU528Po01 CLIA Kit for Argininosuccinate Synthetase 1 (ASS1) CLIA assay for Antigen Detection
ELU528Po01 ELISA Kit for Argininosuccinate Synthetase 1 (ASS1) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Bos taurus; Bovine (Cattle)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPU528Bo01 Recombinant Argininosuccinate Synthetase 1 (ASS1) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAU528Bo01 Monoclonal Antibody to Argininosuccinate Synthetase 1 (ASS1) WB; IHC; ICC; IP.
PAU528Bo01 Polyclonal Antibody to Argininosuccinate Synthetase 1 (ASS1) WB; IHC; ICC; IF.
Assay Kits CLU528Bo01 CLIA Kit for Argininosuccinate Synthetase 1 (ASS1) CLIA assay for Antigen Detection
ELU528Bo01 ELISA Kit for Argininosuccinate Synthetase 1 (ASS1) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Oryctolagus cuniculus (Rabbit)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPU528Rb01 Recombinant Argininosuccinate Synthetase 1 (ASS1) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAU528Rb01 Monoclonal Antibody to Argininosuccinate Synthetase 1 (ASS1) WB; IHC; ICC; IP.
PAU528Rb01 Polyclonal Antibody to Argininosuccinate Synthetase 1 (ASS1) WB; IHC; ICC; IF.
Assay Kits CLU528Rb01 CLIA Kit for Argininosuccinate Synthetase 1 (ASS1) CLIA assay for Antigen Detection
ELU528Rb01 ELISA Kit for Argininosuccinate Synthetase 1 (ASS1) Enzyme-linked immunosorbent assay for Antigen Detection

Organism species: Rhesus monkey (Simian)

PRODUCT TYPE CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPU528Mo01 Recombinant Argininosuccinate Synthetase 1 (ASS1) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies MAU528Mo01 Monoclonal Antibody to Argininosuccinate Synthetase 1 (ASS1) WB; IHC; ICC; IP.
PAU528Mo01 Polyclonal Antibody to Argininosuccinate Synthetase 1 (ASS1) WB; IHC; ICC; IF.
Assay Kits CLU528Mo01 CLIA Kit for Argininosuccinate Synthetase 1 (ASS1) CLIA assay for Antigen Detection
ELU528Mo01 ELISA Kit for Argininosuccinate Synthetase 1 (ASS1) Enzyme-linked immunosorbent assay for Antigen Detection

Reference

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Nucleic Acids Res. 11:6505-6512(1983) [PubMed] [Europe PMC] [Abstract]

"Molecular structures of human argininosuccinate synthetase pseudogenes. Evolutionary and mechanistic implications."
J. Biol. Chem. 259:3160-3166(1984) [PubMed] [Europe PMC] [Abstract]

"Structure of the human argininosuccinate synthetase gene and an improved system for molecular diagnostics in patients with classical and mild citrullinemia."
Hum. Genet. 110:327-333(2002) [PubMed] [Europe PMC] [Abstract]

"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]

"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]

"Structure of the 5' end region of the human argininosuccinate synthetase gene."
J. Inherit. Metab. Dis. 8:157-159(1985) [PubMed] [Europe PMC] [Abstract]

"Identification of essential arginine residue(s) for Mg-ATP binding of human argininosuccinate synthetase."
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"A two-dimensional gel database of human colon carcinoma proteins."
Electrophoresis 18:605-613(1997) [PubMed] [Europe PMC] [Abstract]

"Restructuring of the dinucleotide-binding fold in an NADP(H) sensor protein."
Proc. Natl. Acad. Sci. U.S.A. 104:8809-8814(2007) [PubMed] [Europe PMC] [Abstract]

"Quantitative phosphoproteomics reveals widespread full phosphorylation site occupancy during mitosis."
Sci. Signal. 3:RA3-RA3(2010) [PubMed] [Europe PMC] [Abstract]

"Initial characterization of the human central proteome."
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]

"An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]

"Structure of human argininosuccinate synthetase."
Acta Crystallogr. D 64:279-286(2008) [PubMed] [Europe PMC] [Abstract]

"Mutations and polymorphisms in the human argininosuccinate synthetase (ASS1) gene."
Hum. Mutat. 30:300-307(2009) [PubMed] [Europe PMC] [Abstract]

"Heterogeneity of mutations in argininosuccinate synthetase causing human citrullinemia."
J. Biol. Chem. 265:11361-11367(1990) [PubMed] [Europe PMC] [Abstract]

"Additional mutations in argininosuccinate synthetase causing citrullinemia."
Mol. Biol. Med. 8:95-100(1991) [PubMed] [Europe PMC] [Abstract]

"Mutations in argininosuccinate synthetase mRNA of Japanese patients, causing classical citrullinemia."
Am. J. Hum. Genet. 55:1103-1112(1994) [PubMed] [Europe PMC] [Abstract]

"Characterization of human wild-type and mutant argininosuccinate synthetase proteins expressed in bacterial cells."
Enzyme Protein 48:251-264(1994) [PubMed] [Europe PMC] [Abstract]

"Phenotype and genotype heterogeneity in Mediterranean citrullinemia."
Mol. Genet. Metab. 74:396-398(2001) [PubMed] [Europe PMC] [Abstract]

"Identification of 16 novel mutations in the argininosuccinate synthetase gene and genotype-phenotype correlation in 38 classical citrullinemia patients."
Hum. Mutat. 22:24-34(2003) [PubMed] [Europe PMC] [Abstract]

"Mild citrullinemia in Caucasians is an allelic variant of argininosuccinate synthetase deficiency (citrullinemia type 1)."
Mol. Genet. Metab. 80:302-306(2003) [PubMed] [Europe PMC] [Abstract]

"Prenatal diagnosis of citrullinemia and argininosuccinic aciduria: evidence for a transmission ratio distortion in citrullinemia."
Prenat. Diagn. 26:242-247(2006) [PubMed] [Europe PMC] [Abstract]

"Functional analysis of novel splicing and missense mutations identified in the ASS1 gene in classical citrullinemia patients."
Clin. Chim. Acta 438:323-329(2015) [PubMed] [Europe PMC] [Abstract]