Western Blot (WB)
(WB Suggested Anti-NAGS Antibody Titration: 1.25ug/mlPositive Control: HepG2 cell lysate)
Rabbit NAGS Polyclonal Antibody | anti-NAGS antibody
NAGS antibody - C-terminal region
Western Blot (WB)
(WB Suggested Anti-NAGS Antibody Titration: 1.25ug/mlPositive Control: HepG2 cell lysate)
Western Blot (WB)
(WB Suggested Anti-NAGS Antibody Titration: 1.25ug/mlPositive Control: HepG2 cell lysate)
Target Description: NAGS is a mitochondrial enzyme that catalyzes the formation of N-acetylglutamate (NAG) from glutamate and acetyl coenzyme-A. NAG is a cofactor of carbamyl phosphate synthetase I (CPSI), the first enzyme of the urea cycle in mammals. Deficiencies in N-acetylglutamate synthase have been associated with hyperammonemia.The N-acetylglutamate synthase gene encodes a mitochondrial enzyme that catalyzes the formation of N-acetylglutamate (NAG) from glutamate and acetyl coenzyme-A. NAG is a cofactor of carbamyl phosphate synthetase I (CPSI), the first enzyme of the urea cycle in mammals. This gene may regulate ureagenesis by altering NAG availability and, thereby, CPSI activity. Deficiencies in N-acetylglutamate synthase have been associated with hyperammonemia.
NCBI and Uniprot Product Information
NCBI Description
The N-acetylglutamate synthase gene encodes a mitochondrial enzyme that catalyzes the formation of N-acetylglutamate (NAG) from glutamate and acetyl coenzyme-A. NAG is a cofactor of carbamyl phosphate synthetase I (CPSI), the first enzyme of the urea cycle in mammals. This gene may regulate ureagenesis by altering NAG availability and, thereby, CPSI activity. Deficiencies in N-acetylglutamate synthase have been associated with hyperammonemia. [provided by RefSeq, Jul 2008]
Uniprot Description
NAGS: Plays a role in the regulation of ureagenesis by producing variable amounts of N-acetylglutamate (NAG), thus modulating carbamoylphosphate synthase I (CPSI) activity. Defects in NAGS are the cause of N-acetylglutamate synthase deficiency (NAGSD). NAGSD is a rare autosomal recessively inherited metabolic disorder leading to severe neonatal or late onset hyperammonemia without increased excretion of orotic acid. Clinical symptoms are somnolence, tachypnea, feeding difficulties, a severe neurologic presentation characterized by uncontrollable movements, developmental delay, visual impairment, failure to thrive and hyperammonemia precipitated by the introduction of high-protein diet or febrile illness. Belongs to the acetyltransferase family.
Protein type: Acetyltransferase; Mitochondrial; Amino Acid Metabolism - arginine and proline; EC 2.3.1.1
Chromosomal Location of Human Ortholog: 17q21.31
Cellular Component: mitochondrial matrix
Molecular Function: amino-acid N-acetyltransferase activity
Biological Process: glutamate metabolic process; arginine biosynthetic process; urea cycle
Disease: N-acetylglutamate Synthase Deficiency
Research Articles on NAGS
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Product Notes
The NAGS nags (Catalog #AAA3210236) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The NAGS antibody - C-terminal region reacts with Cow, Dog, Guinea Pig, Horse, Human, Mouse, Pig, Rabbit, Rat, Zebrafish and may cross-react with other species as described in the data sheet. AAA Biotech's NAGS can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Researchers should empirically determine the suitability of the NAGS nags 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. The amino acid sequence is listed below: Synthetic peptide located within the following region: YLDKFVVSSS RQGQGSGQML WECLRRDLQT LFWRSRVTNP INPWYFKHSD. It is sometimes possible for the material contained within the vial of "NAGS, 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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