Western Blot (WB)
(Western blot analysis of ASS1 expression in HeLa (A), mouse heart (B), rat kidney (C) whole cell lysates.)
Rabbit ASS1 Polyclonal Antibody | anti-ASS1 antibody
Anti-ASS1 Antibody
Western Blot (WB)
(Western blot analysis of ASS1 expression in HeLa (A), mouse heart (B), rat kidney (C) whole cell lysates.)
Western Blot (WB)
(Western blot analysis of ASS1 expression in HeLa (A), mouse heart (B), rat kidney (C) whole cell lysates.)
NCBI and Uniprot Product Information
NCBI Description
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 Description
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 adulthood. Belongs to the argininosuccinate synthase family. Type 1 subfamily.
Protein type: Amino Acid Metabolism - arginine and proline; Amino Acid Metabolism - alanine, aspartate and glutamate; Mitochondrial; EC 6.3.4.5; Endoplasmic reticulum; Ligase
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; arginine biosynthetic process; response to mycotoxin; 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; urea cycle; aging
Disease: Citrullinemia, Classic
Research Articles on ASS1
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Product Notes
The ASS1 ass1 (Catalog #AAA8306072) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Anti-ASS1 Antibody reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's ASS1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). WB: 1:500-1:2000. Researchers should empirically determine the suitability of the ASS1 ass1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "ASS1, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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