Product Name
Argininosuccinate Synthetase 1 (ASS1), Enzyme
Full Product Name
Argininosuccinate Synthetase 1 (ASS1, CTLN1, Citrulline-aspartate ligase) (Control Peptide)
Product Gene Name
ASS1 enzyme
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Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Chromosome Location
Chromosome: 9; NC_000009.11 (133320094..133376661). Location: 9q34.1
3D Structure
ModBase 3D Structure for P00966
Purity/Purification
Highly Purified
Highly Purified
Form/Format
Supplied as a lyophilized powder in PBS.
Preparation and Storage
May be stored at 4 degree C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and store at -20 degree C. Aliquots are stable for at least 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
Other Notes
Small volumes of ASS1 enzyme vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
ASS1 enzyme
C-terminus of aa-ENPKNQAPPGLYTKTQD
Product Categories/Family for ASS1 enzyme
Molecular Biology; MB-Enzymes, Synthetase
Applications Tested/Suitable for ASS1 enzyme
ELISA (EL/EIA)
Application Notes for ASS1 enzyme
Suitable for use in ELISA.
NCBI/Uniprot data below describe general gene information for ASS1. It may not necessarily be applicable to this product.
NCBI Accession #
NP_000041.2
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NCBI GenBank Nucleotide #
NM_000050.4
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UniProt Primary Accession #
P00966
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UniProt Secondary Accession #
Q6LDL2; Q86UZ0; Q96GT4[Other Products]
UniProt Related Accession #
P00966[Other Products]
Molecular Weight
46,530 Da[Similar Products]
NCBI Official Full Name
argininosuccinate synthase
NCBI Official Synonym Full Names
argininosuccinate synthase 1
NCBI Official Symbol
ASS1??[Similar Products]
NCBI Official Synonym Symbols
ASS; CTLN1
??[Similar Products]
NCBI Protein Information
argininosuccinate synthase; citrulline-aspartate ligase; citrulline--aspartate ligase; argininosuccinate synthetase 1
UniProt Protein Name
Argininosuccinate synthase
UniProt Synonym Protein Names
Citrulline--aspartate ligase
UniProt Gene Name
ASS1??[Similar Products]
UniProt Synonym Gene Names
ASS??[Similar Products]
UniProt Entry Name
ASSY_HUMAN
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
Function: Is indirectly involved in the control of blood pressure
By similarity.
Catalytic activity: ATP + L-citrulline + L-aspartate = AMP + diphosphate + N(omega)-(L-arginino)succinate.
Pathway: Amino-acid biosynthesis; L-arginine biosynthesis; L-arginine from L-ornithine and carbamoyl phosphate: step 2/3.Nitrogen metabolism; urea cycle; (N(omega)-L-arginino)succinate from L-aspartate and L-citrulline: step 1/1.
Subunit structure: Homotetramer. Interacts with NMRAL1. Ref.9 Ref.12
Involvement in disease: Citrullinemia 1 (CTLN1) [MIM:215700]: 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 *****hood.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.3 Ref.14 Ref.15 Ref.16 Ref.17 Ref.18 Ref.19 Ref.20 Ref.21
Sequence similarities: Belongs to the argininosuccinate synthase family. Type 1 subfamily.
Research Articles on ASS1
1. ASS expression is decreased significantly in hepatocellular carcinoma tissues.
Precautions
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Disclaimer
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