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

E.coli

> 90%

20mM Tris, 150mM NaCl, pH8.0, containing 1mM EDTA, 1mM DTT, 0.01% SKL, 5% Trehalose and Proclin300.

Original Concentration: 200ug/mL (lot specific)

Storage: Avoid repeated freeze/thaw cycles.
Store at 2-8oC for one month.
Aliquot and store at -80oC for 12 months.

Stability Test: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

Small volumes of ASS1 recombinant protein 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.

Positive Control; Immunogen; SDS-PAGE; Western Blot (WB)

(May be suitable for use in other assays to be determined by the end user.)

Predicted Molecular Mass: 50.2kDa
Accurate Molecular Mass: 47kDa as determined by SDS-PAGE reducing conditions.

argininosuccinate synthase 1

argininosuccinate synthase

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 this gene cause citrullinemia. Two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Aug 2012]

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

Chromosomal Location of Human Ortholog: 9q34.1

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

Molecular Function: toxin binding; amino acid 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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