CPS1 Rabbit Polyclonal Antibody

CAT#: TA375046

CPS1 Rabbit polyclonal Antibody

Specifications

Product Data

• Applications: IF, WB
• Recommended Dilution: WB,1:1000 - 1:2000
  IF,1:50 - 1:200
• Reactivities: Human, Mouse, Rat
• Modifications: Unmodified
• Host: Rabbit
• Isotype: IgG
• Clonality: Polyclonal
• Immunogen: Recombinant fusion protein containing a sequence corresponding to amino acids 820-930 of human CPS1 (NP_001116105.1).
• Formulation: Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3.
• Concentration: lot specific
• Purification: Affinity purification
• Conjugation: Unconjugated
• Stability: Shelf life: one year from despatch.
• Predicted Protein Size: 116kDa/164kDa/165kDa
• Gene Name: carbamoyl-phosphate synthase 1
• Database Link:
  Entrez Gene 1373 Human
  UniProt ID P31327
• 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.