Oithine transcarbamylase (OTC) (also called oithine carbamoyltransferase) is an enzyme that catalyzes the reaction between carbamoyl phosphate (CP) and oithine (O) to form citrulline (Cit) and phosphate (Pi). This nuclear gene encodes a mitochondrial matrix enzyme. Missense, nonsense, and frameshift mutations in this enzyme lead to oithine transcarbamylase deficiency, which causes hyperammonemia. Since the gene for this enzyme maps close to that for Duchenne muscular dystrophy, it may also play a role in that disease.
Formulation
0.5mg/ml if reconstituted with 0.2ml sterile DI water
Host
Rabbit
Immunogen Region
Amino acids 33-70 (NKVQLKGRDLLTLKNFTGEEIKYMLWLSADLKFRIKQK) from the human protein were used as the immunogen for the OTC antibody.
Isotype
IgG
Predicted Reactivity
Mouse, Rat
Reactivity
Mouse, Rat
Recombinant
No
Uniprot
P00480
Buffer
Lyophilized from 1X PBS with 2.5% BSA, 0.025% sodium azide
Format
Antigen affinity purified
Purification
Antigen affinity
Storage
After reconstitution, the OTC antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.