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The mitochondrial enzyme encoded by CPS1 catalyzes synthesis of carbamoyl phosphate from ammonia and bicarbonate. Additionally we are shipping CPS1 Kits (3) and CPS1 Proteins (3) and many more products for this protein.
Showing 10 out of 113 products:
Human Polyclonal CPS1 Primary Antibody for EIA, WB - ABIN951681
Pekkala, Martínez, Barcelona, Yefimenko, Finckh, Rubio, Cervera: Understanding carbamoyl-phosphate synthetase I (CPS1) deficiency by using expression studies and structure-based analysis. in Human mutation 2010
Show all 5 references for ABIN951681
Chicken Polyclonal CPS1 Primary Antibody for IHC, WB - ABIN2782318
Huo, Zhu, Lu, Ying, Xu, Xu, Li, Zhou, Sha: Molecular cloning, identification and characteristics of a novel isoform of carbamyl phosphate synthetase I in human testis. in Journal of biochemistry and molecular biology 2005
Human Monoclonal CPS1 Primary Antibody for IHC (p), ELISA - ABIN560455
Lee, Jee, Kwon, Yoon, Xu, Wang, Wang, Thorgeirsson, Lee, Woo, Yoon: Identification of a mitochondrial defect gene signature reveals NUPR1 as a key regulator of liver cancer progression. in Hepatology (Baltimore, Md.) 2015
Molecular structure of CPS1 has been deciphered.
CPS1 and CPS1IT1 may be potential prognostic indicators for patients with intrahepatic cholangiocarcinoma.
CPS1 is involved in the urea cycle in weight maintenance.
More HCC (show FAM126A Antibodies) cells could be identified by the antibody cocktail for CPS1 and P-CK compared with a single antibody.
characterized the only currently known recurrent CPS1 mutation, p.Val1013del found in eleven unrelated patients of Turkish descent; mutation p.V1013del inactivates CPS1 but does not render the enzyme grossly unstable or insoluble
Overexpression of CPS1 is associated with rectal cancers.
study examined patient characteristics, including genetic polymorphism, to identify risk factors associated with development of hyperammonemia during valproic acid-based therapy; found CPS1 4217C>A polymorphism may not be associated with development of hyperammonemia in Japanese population
Findings support the disease-causing role of the mutations reported to affect the CPS1 deficiency, revealing a key role of the small CPS1 domain of unknown function (UFSD) for proper enzyme folding.
CPS1 becomes readily detectable upon hepatocyte apoptotic and necrotic death. Its abundance and short serum half-life suggest that it may be a useful prognostic biomarker in acute liver injury.
Data show that carbamoyl phosphate synthetase 1, an enzyme involved in the urea cycle, 8-oxoguanine DNA glycosylase 1 (show OGG1 Antibodies) and DNA polymerase beta (show POLB Antibodies), enzymes involved in DNA repair, were expressed at higher levels in Batten disease cells than in normal cells.
these data suggest that AhR (show AHR Antibodies) activation promotes CPS1 recruitment to DNA enhancer sites in the genome, resulting in a specific enzyme-independent post-translational modification of the linker histone H1 (show H1F0 Antibodies) protein (H1K34hcit), pivotal in altering local chromatin structure and transcriptional activation.
The results suggest that elevated isoaspartate and CPS-1, and reduced CA-III (show CA3 Antibodies) levels could serve as biomarkers of hepatocellular injury.
Taken together with previous findings regarding CPS1 structure and function, homology modeling of mouse CPS1 suggested that nitration at Y1450 in an alpha-helix of allosteric domain prevents activation of CPS1 by its activator, N-acetyl-l-glutamate (show GRIN1 Antibodies).
Because ammonia generated during fasting is toxic, SIRT5 (show SIRT5 Antibodies) protein might play a protective role by converting ammonia to non-toxic urea through deacetylation and activation of CPS1.
Y-box binding protein-1 (show YBX1 Antibodies) down-regulates expression of carbamoyl phosphate synthetase-I by suppressing CCAAT enhancer-binding protein-alpha (show CEBPA Antibodies) function in mice.
SIRT5 (show SIRT5 Antibodies) deacetylates CPS1 and upregulates its activity
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.
carbamoyl-phosphate synthase [ammonia], mitochondrial
, carbamoyl-phosphate synthetase III
, carbamoyl-phosphate synthase 1, mitochondrial
, carbamoyl-phosphate synthetase 1, mitochondrial
, carbamoyl-phosphate synthase [ammonia], mitochondrial-like
, carbamoyl-phosphate synthetase I
, carbamoyl phosphate synthetase 1
, carbamyl phosphate synthetase 1
, carbamoylphosphate synthetase I
, CPSase I
, carbamyl phosphate synthetase I
, carboamyl-phosphate synthetase 1