Western Blot (WB)
(Western Blot: Sample: Recombinant ASS1, Human.)
Rabbit anti-Human Argininosuccinate Synthetase 1 Polyclonal Antibody | anti-ASS1 antibody
Polyclonal Antibody to Argininosuccinate Synthetase 1 (ASS1)
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 (WB)
(Western Blot: Sample: Recombinant ASS1, Human.)
Western Blot (WB)
(Western Blot: Sample: Recombinant ASS1, Human.)
Immunohistochemistry (IHC)
(DAB staining on IHC-P; Samples: Human Kidney Tissue.)
Immunohistochemistry (IHC)
(DAB staining on IHC-P; Samples: Human Kidney Tissue.)
Immunohistochemistry (IHC)
(DAB staining on IHC-P; Samples: Human Kidney Tissue))
Immunohistochemistry (IHC)
(DAB staining on IHC-P; Samples: Human Kidney Tissue))
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 - 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
Research Articles on ASS1
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Product Notes
The ASS1 ass1 (Catalog #AAA2026755) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Polyclonal Antibody to Argininosuccinate Synthetase 1 (ASS1) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's Argininosuccinate Synthetase 1 can be used in a range of immunoassay formats including, but not limited to, 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. 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 "Argininosuccinate Synthetase 1, 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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