Optimal dilution of the OTC antibody should be determined by the researcher.\. WB: 0.5-1 μg/mL
限制
仅限研究用
缓冲液
0.5 mg/mL if reconstituted with 0.2 mL sterile DI water
储存条件
-20 °C
储存方法
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.
Ornithine transcarbamylase (OTC) (also called ornithine carbamoyltransferase) is an enzyme that catalyzes the reaction between carbamoyl phosphate (CP) and ornithine (Orn) 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 ornithine 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.