Product Datasheet  
CPS1 Antibody  
Catalog Number: 46982  
Technical:tech@swbio.com  
Information:info@swbio.com  
Description  
  • host_species：  
  • Rabbit
  • Amount：  
  • 100μgμg
  • Swiss-Prot No.：  
  • Swiss-Prot#:P31327
    NCBI Gene ID:1373
    Gene Accssion:NP_001866
  • Form of Antibody：  
  • Rabbit IgG in pH7.4 PBS, 0.05% NaN3, 40% Glycerol.
  • Storage：  
  • Store at -20C
  • Immunogen：  
  • Synthetic peptide of human CPS1
  • reactivity：  
  • Hu
  • appl_detail：  

  • Immunofluorescence:1: 25-100
  • other_names：  
  • PHN; CPSASE1
  • Purification：  
  • Antigen affinity purification
  • Specificity：  
  • The antibody detects endogenous levels of total CPS1 protein.
  • Applications：  
  • IHC
  • Background：  
  • 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.



 
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