NP_000041.2
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Related Accession #
NCBI Official Full Name
argininosuccinate synthase
NCBI Official Synonym Full Names
argininosuccinate synthase 1
NCBI Official Synonym Symbols
NCBI Protein Information
argininosuccinate synthase
UniProt Protein Name
Argininosuccinate synthase
NCBI Summary for ASS1
The protein encoded by this gene 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. Mutations in the chromosome 9 copy of this gene cause citrullinemia. Two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Aug 2012]
UniProt Comments for 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.
Research Articles on ASS1
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Products associated with anti-ASS1 antibody
Pathways associated with anti-ASS1 antibody
Diseases associated with anti-ASS1 antibody
Organs/Tissues associated with anti-ASS1 antibody
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