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

Polyclonal

IgG

Rabbit

The antibody is a rabbit polyclonal antibody raised against ASS1. It has beenselected for its ability to recognize ASS1 in immunohistochemical staining andwestern blotting.

Affinity Chromatography

Liquid

200ug/ml (lot specific)

Store at 4 degree C for frequent use. Stored at -20 degree C to -80 degree C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

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 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.

Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (EIA)

Western blotting: 0.5-2ug/ml
Immunocytochemistry in formalin fixed cells: 5-20ug/ml
Immunohistochemistry in formalin fixed frozen section: 5-20ug/ml
Immunohistochemistry in paraffin section: 5-20ug/ml
Enzyme-linked Immunosorbent Assay: 0.05-2ug/ml
Optimal working dilutions must be determined by end user.

Western Blot: Sample: Recombinant ASS1, Human.

DAB staining on IHC-P; Samples: Human Kidney Tissue.

DAB staining on IHC-P; Samples: Human Kidney Tissue)

46,530 Da

argininosuccinate synthase 1

argininosuccinate synthase

Argininosuccinate synthase

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

Chromosomal Location of Human Ortholog: 9q34.11

Cellular Component: cytoplasm; cytosol; mitochondrion; myelin sheath

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

Biological Process: arginine biosynthetic process; argininosuccinate metabolic process; aspartate metabolic process; citrulline metabolic process; positive regulation of nitric oxide biosynthetic process; urea cycle

Disease: Citrullinemia, Classic

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