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产品性质
biological source【生物来源】 | rabbit |
Quality Level【质量水平】 | 100 |
antibody product type | primary antibodies |
clone【克隆】 | monoclonal |
form【形式】 | liquid |
species reactivity | rat, human |
concentration【浓度】 | 0.33 mg/mL |
technique(s) | immunohistochemistry: 1:50-1:200 western blot: 1:500-1:2000 |
UniProt accession no.【UniProt登记号】 | P31327 |
shipped in【运输】 | wet ice |
storage temp.【储存温度】 | −20℃ |
Gene Information | human ... CPS1(1373) |
基本信息
General description【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] |
Immunogen【免疫原】 | A synthesized peptide derived from human CPS1 |
Physical form【外形】 | Buffer: PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3. |