Anti -Argininosuccinate Synthetase 1 (ASS1, CTLN1, Citrulline-aspartate ligase)

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 9; NC_000009.11 (133320094..133376661). Location: 9q34.1

Polyclonal

Goat

Recognizes human Argininosuccinate synthetase 1. Species Crossreactivity: mouse, rat, canine

Affinity Purified
Purified by affinity chromatography.

Supplied as a liquid in Tris saline, pH 7.2, 0.5% BSA, 0.02% sodium azide.

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.

Small volumes of anti-ASS1 antibody 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.

Antibodies; Abs to Enzymes, Synthetase

ELISA (EL/EIA), Western Blot (WB), Immunoprecipitation (IP)

Suitable for use in ELISA, Western Blot, Immunoprecipitation.
Dilution: Peptide ELISA: 1:64,000.
Western Blot: 0.01-0.03ug/ml. A ~45kD band is observed in Human Kidney lysates.

46,530 Da[Similar Products]

argininosuccinate synthase 1

argininosuccinate synthase; OTTHUMP00000022362; OTTHUMP00000022363; OTTHUMP00000022364; citrulline-aspartate ligase; citrulline--aspartate ligase; argininosuccinate synthetase 1

Argininosuccinate synthase

ASS??[Similar Products]

ASSY_HUMAN

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 ASS cause citrullinemia. Two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq]

ASS1: Defects in ASS1 are the cause of citrullinemia type 1 (CTLN1). Citrullinemia belongs to the urea cycle disorders. It is an autosomal recessive disease characterized primarily by elevated serum and urine citrulline levels. Ammonia intoxication is another manifestation. CTLN1 usually manifests 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 CTLN1 form can develop later in childhood or *****hood. Belongs to the argininosuccinate synthase family. Type 1 subfamily.

Protein type: Amino Acid Metabolism - arginine and proline; Ligase; EC 6.3.4.5; Amino Acid Metabolism - alanine, aspartate and glutamate; Mitochondrial; Endoplasmic reticulum

Chromosomal Location of Human Ortholog: 9q34.1

Cellular Component: mitochondrial outer membrane; lysosome; endoplasmic reticulum; cytoplasm; perikaryon; nucleus; cytosol

Molecular Function: amino acid binding; toxin binding; protein binding; argininosuccinate synthase activity; ATP binding

Biological Process: response to drug; positive regulation of nitric oxide biosynthetic process; response to mycotoxin; arginine biosynthetic process; citrulline metabolic process; liver development; response to estradiol stimulus; response to zinc ion; acute-phase response; midgut development; aspartate metabolic process; kidney development; argininosuccinate metabolic process; response to nutrient; aging; urea cycle

Disease: Citrullinemia, Classic

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