Human Carbamoyl-phosphate synthase [ammonia], mitochondrial(CPS1) ELISA kit

Code CSB-EL005913HU
Size 96T,5×96T,10×96T
Trial Size 24T ELISA kits trial application
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Product Details

Target Name carbamoyl-phosphate synthetase 1, mitochondrial
Alternative Names Carbamoyl phosphate synthase [ammonia] ELISA Kit; Carbamoyl phosphate synthase [ammonia] mitochondrial ELISA Kit; Carbamoyl phosphate synthase ELISA Kit; Carbamoyl phosphate synthetase 1 ELISA Kit; Carbamoyl phosphate synthetase 1 mitochondrial ELISA Kit; Carbamoyl phosphate synthetase I ELISA Kit; Carbamoyl-phosphate synthase [ammonia] ELISA Kit; Carbamoyl-phosphate synthetase I ELISA Kit; Carbamoylphosphate synthase ELISA Kit; Carbamoylphosphate synthetase 1 ELISA Kit; Carbamoylphosphate synthetase I ELISA Kit; CPS 1 ELISA Kit; Cps1 ELISA Kit; CPSase 1 ELISA Kit; CPSase I ELISA Kit; CPSASE1 ELISA Kit; CPSM_HUMAN ELISA Kit; mitochondrial ELISA Kit; MS738 ELISA Kit
Abbreviation CPS1
Uniprot No. P31327
Species Homo sapiens (Human)
Sample Types serum, plasma, tissue homogenates
Detection Range 78 pg/mL-5000 pg/mL
Sensitivity 19.5 pg/mL
Assay Time 1-5h
Sample Volume 50-100ul
Detection Wavelength 450 nm
Research Area Metabolism
Assay Principle quantitative
Measurement Sandwich
Intra-assay Precision (Precision within an assay): CV%<8%      
Three samples of known concentration were tested twenty times on one plate to assess.  
Inter-assay Precision (Precision between assays): CV%<10%      
Three samples of known concentration were tested in twenty assays to assess.    
To assess the linearity of the assay, samples were spiked with high concentrations of human CPS1 in various matrices and diluted with the Sample Diluent to produce samples with values within the dynamic range of the assay.
  Sample Serum(n=4)  
1:1 Average % 96  
Range % 90-102  
1:2 Average % 100  
Range % 93-107  
1:4 Average % 99  
Range % 94-102  
1:8 Average % 89  
Range % 83-95  
The recovery of human CPS1 spiked to levels throughout the range of the assay in various matrices was evaluated. Samples were diluted prior to assay as directed in the Sample Preparation section.
Sample Type Average % Recovery Range  
Serum (n=5) 89 85-93  
EDTA plasma (n=4) 96 90-102  
Typical Data
These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed.
pg/ml OD1 OD2 Average Corrected  
5000 2.306 2.367 2.337 2.191  
2500 2.171 2.191 2.181 2.035  
1250 1.807 1.847 1.827 1.681  
625 1.393 1.371 1.382 1.236  
312.5 0.801 0.824 0.813 0.667  
156 0.552 0.591 0.572 0.426  
78 0.362 0.371 0.367 0.221  
0 0.143 0.149 0.146    
and FAQs
Storage Store at 2-8°C. Please refer to protocol.
Lead Time 5-7 working days

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Target Data

Function Involved in the urea cycle of ureotelic animals where the enzyme plays an important role in removing excess ammonia from the cell.
Gene References into Functions
  1. In silico analysis potentially links CPS1 SNPs with major depression disorder. PMID: 29441491
  2. Allele and genotype frequencies of the p.Thr1406Asn polymorphism did not differ between the infants with and without NEC, but the minor A-allele was less frequent in the group of 64 infants with the combined outcome NEC or death before 34 weeks of corrected gestational age than in the infants without this outcome. A significant negative association of the A-allele with the combined outcome NEC or death was found. PMID: 27833157
  3. HNF3beta plays a vital role in regulation of CPS1 gene and could promote the metabolism of ammonia by regulating CPS1 expression. PMID: 28272778
  4. CPS1 maintains pyrimidine pools and DNA synthesis in KRAS/LKB1-mutant lung cancer cells PMID: 28538732
  5. CPS1 knockdown reduced cell growth, decreased levels of metabolites associated with nucleic acid biosynthesis. PMID: 28376202
  6. These results may offer an increasing understand that CPS1 might have a function in differentiation. PMID: 27425868
  7. Molecular structure of CPS1 has been deciphered. PMID: 26592762
  8. CPS1 and CPS1IT1 may be potential prognostic indicators for patients with intrahepatic cholangiocarcinoma. PMID: 26499888
  9. CPS1 is involved in the urea cycle in weight maintenance. PMID: 26938218
  10. These results suggest that glycine metabolism and/or the urea cycle represent potentially novel sex-specific mechanisms for the development of atherosclerosis. PMID: 26822151
  11. Mechanism for Switching On/Off the Urea Cycle PMID: 26059772
  12. More HCC cells could be identified by the antibody cocktail for CPS1 and P-CK compared with a single antibody. PMID: 24763545
  13. 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 PMID: 25410056
  14. Overexpression of CPS1 is associated with rectal cancers. PMID: 25099619
  15. 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 PMID: 24888247
  16. 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. PMID: 24813853
  17. 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. PMID: 24924744
  18. Data show that carbamoyl phosphate synthetase 1, an enzyme involved in the urea cycle, 8-oxoguanine DNA glycosylase 1 and DNA polymerase beta, enzymes involved in DNA repair, were expressed at higher levels in Batten disease cells than in normal cells. PMID: 22692827
  19. the human CPS unknown function domains are spatially located in a region that corresponds to the a/b subunits interface in Escherichia coli CPS. [Review] PMID: 22521883
  20. Mutation analysis in these patients identified 17 genetic lesions, 9 of which were new confirming their "private" nature. PMID: 22173106
  21. Case Report: Late-onset carbamoyl phosphate synthetase 1 deficiency in an adult cured by liver transplantation. PMID: 21837743
  22. This is the first large-scale report of CPS1 mutations spanning a wide variety of molecular defects highlighting important regions in this protein. PMID: 21120950
  23. DNA methylation is a key mechanism of silencing CPS1 expression in human hepatocellular carcinoma cells. PMID: 21281797
  24. structure-function analysis and pathogenicity-testing of mutations in CPS1 PMID: 20800523
  25. Data reported five of the CPS1 mutations (p.T471N, p.Q678P, p.P774L, p.R1453Q, and p.R1453W) in severe CPS1D patients. PMID: 20578160
  26. The present study in preterm infants did not confirm the earlier reported association between CPS1 genotype and L-arginine levels in term infants. PMID: 20520828
  27. These data confirm a recent finding that CPS1 is a locus influencing homocysteine levels in women and suggest that genetic effects on Hcy may differ across developmental stages. PMID: 20154341
  28. Meta-analysis and genome-wide association study of gene-disease association. (HuGE Navigator) PMID: 20383146
  29. Observational study and genome-wide association study of gene-disease association and gene-gene interaction. (HuGE Navigator) PMID: 20154341
  30. CPS1, MUT, NOX4, and DPEP1 is associated with plasma homocysteine in healthy Women. PMID: 20031578
  31. Allelic imbalance may explain clinical variability in CPS1 deficiency in some families. PMID: 19793055
  32. The entire DNA sequence of the human CPS1 gene is presented, including all exon-intron boundaries. PMID: 12655559
  33. CPSI T1405N genotype appears to be an important new factor in predicting susceptibility to pulmonry hypertension following surgical repair of congenital cardiac defects in children. PMID: 17188582
  34. CPPS1 T1404N polymorphism may be associated with the risk of necrotizing enterocolitis in preterm infants. PMID: 17597649
  35. Tight hydrogen binding mode is supported by the observation of reduced NAG affinity upon mutation of N-acetyl-L-glutamate-interacting residues of CPSI PMID: 19754428
  36. The differential expression of Hep Par 1(carbamoyl phosphate synthetase I) in dysplastic vs malignant tumors of the small intestine may be diagnostically useful in difficult cases. PMID: 19926579

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Involvement in disease Carbamoyl phosphate synthetase 1 deficiency (CPS1D); Pulmonary hypertension, neonatal (PHN)
Subcellular Location Mitochondrion, Nucleus, nucleolus
Tissue Specificity Primarily in the liver and small intestine.
Database Links

HGNC: 2323

OMIM: 237300

KEGG: hsa:1373

STRING: 9606.ENSP00000402608

UniGene: Hs.149252


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