产品说明
一般描述
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.[provided by RefSeq, May 2010]
免疫原
A synthesized peptide derived from human CPS1
外形
Buffer: PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.
产品性质
生物来源 | rabbit |
质量水平 | 100 |
antibody product type | primary antibodies |
克隆 | monoclonal |
形式 | liquid |
species reactivity | rat, human |
浓度 | 0.33 mg/mL |
technique(s) | immunohistochemistry: 1:50-1:200 western blot: 1:500-1:2000 |
UniProt登记号 | P31327 |
运输 | wet ice |
储存温度 | −20℃ |
Gene Information | human ... CPS1(1373) |
安全信息
闪点(F) | Not applicable |
闪点(C) | Not applicable |
Sigma-Aldrich