Western Blot (WB)
(CPS1 Antibody (N-term) western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the CPS1 antibody detected the CPS1 protein (arrow).)
Rabbit anti-Human CPS1 Polyclonal Antibody | anti-CPS1 antibody
CPS1 Antibody (N-term)
Western Blot (WB)
(CPS1 Antibody (N-term) western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the CPS1 antibody detected the CPS1 protein (arrow).)
Western Blot (WB)
(CPS1 Antibody (N-term) western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the CPS1 antibody detected the CPS1 protein (arrow).)
synthesis of carbamoyl phosphate from ammonia and bicarbonate. This
reaction is the first committed step of the urea cycle, which is
important in the removal of excess urea from cells. The encoded
protein may also represent a core mitochondrial nucleoid protein.
Three transcript variants encoding different isoforms have been
found for this gene. The shortest isoform may not be localized to
the mitochondrion. Mutations in this gene have been associated with
carbamoyl phosphate synthetase deficiency, susceptibility to
persistent pulmonary hypertension, and susceptibility to
venoocclusive disease after bone marrow transplantation.
Jia, P., et al. Schizophr. Res. 122 (1-3), 38-42 (2010) :
Pekkala, S., et al. Hum. Mutat. 31(7):801-808(2010)
Huo, R., et al. J. Biochem. Mol. Biol. 38(1):28-33(2005)
Hoshide, R., et al. Genomics 28(1):124-125(1995)
NCBI and Uniprot Product Information
NCBI Description
The mitochondrial enzyme encoded by this gene catalyzes synthesis of carbamoyl phosphate from ammonia and bicarbonate. This reaction is the first committed step of the urea cycle, which is important in the removal of excess urea from cells. The encoded protein may also represent a core mitochondrial nucleoid protein. Three transcript variants encoding different isoforms have been found for this gene. The shortest isoform may not be localized to the mitochondrion. Mutations in this gene have been associated with carbamoyl phosphate synthetase deficiency, susceptibility to persistent pulmonary hypertension, and susceptibility to venoocclusive disease after bone marrow transplantation.[provided by RefSeq, May 2010]
Uniprot Description
CPS1: Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell. Defects in CPS1 are the cause of carbamoyl phosphate synthetase 1 deficiency (CPS1D). CPS1D is an autosomal recessive disorder of the urea cycle causing hyperammonemia. Clinical features include protein intolerance, intermittent ataxia, seizures, lethargy, developmental delay and mental retardation. Genetic variations in CPS1 influence the availability of precursors for nitric oxide (NO) synthesis and play a role in clinical situations where endogenous NO production is critically important, such as neonatal pulmonary hypertension, increased pulmonary artery pressure following surgical repair of congenital heart defects or hepatovenocclusive disease following bone marrow transplantation. Infants with neonatal pulmonary hypertension homozygous for Thr-1406 have lower L-arginine concentrations than neonates homozygous for Asn-1406. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Amino Acid Metabolism - arginine and proline; Amino Acid Metabolism - alanine, aspartate and glutamate; Energy Metabolism - nitrogen; Mitochondrial; Ligase; Nucleolus; EC 6.3.4.16
Chromosomal Location of Human Ortholog: 2q35
Cellular Component: protein complex; mitochondrial matrix; mitochondrial inner membrane; nucleolus
Molecular Function: carbamoyl-phosphate synthase (ammonia) activity; protein binding; glutamate binding; carbamoyl-phosphate synthase (glutamine-hydrolyzing) activity; endopeptidase activity; protein complex binding; phospholipid binding; calcium ion binding; ATP binding
Biological Process: response to food; response to drug; glycogen catabolic process; arginine biosynthetic process; response to toxin; response to lipopolysaccharide; homocysteine metabolic process; response to amino acid stimulus; positive regulation of vasodilation; proteolysis; nitric oxide metabolic process; response to starvation; citrulline biosynthetic process; response to zinc ion; glutamine catabolic process; triacylglycerol catabolic process; midgut development; anion homeostasis; urea cycle; response to amine stimulus
Disease: Pulmonary Hypertension, Neonatal, Susceptibility To; Carbamoyl Phosphate Synthetase I Deficiency, Hyperammonemia Due To
Research Articles on CPS1
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Product Notes
The CPS1 cps1 (Catalog #AAA9213939) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The immunogen sequence is 262-291. The CPS1 Antibody (N-term) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's CPS1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), ELISA (EIA). WB~~1:1000. Researchers should empirically determine the suitability of the CPS1 cps1 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 "CPS1, 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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