Description
Product Name: | CPS1 Polyclonal Antibody |
Product Code: | CAB21361 |
Reactivity: | Human, Rat |
Applications: | Western blotting, Immunofluorescence |
Host Species: | Rabbit |
Purification Method: | Affinity purification |
Isotype: | IgG |
Reactivity: | Human, Rat |
Tested Applications: | WB, IF/ICC, ELISA |
Key Applications: | Western blotting, Immunofluorescence |
Recommended Dilution: | WB 1:100-1:500, IF/ICC 1:50-1:200 |
Storage Buffer: | Store at -20°C. Avoid freeze / thaw cycles.Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Positive Samples: | U2OS(Negative control), Rat liver |
Cellular Location: | Mitochondrion, Nucleus, nucleolus |
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.
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 820-930 of human CPS1 (NP_001116105.1). |
Sequence: | RMCHP SIEGF TPRLP MNKEW PSNLD LRKEL SEPSS TRIYA IAKAI DDNMS LDEIE KLTYI DKWFL YKMRD ILNME KTLKG LNSES MTEET LKRAK EIGFS DKQIS KCLGL T |
Synonyms: | PHN, GATD6, CPSASE1 |
Calculated MW: | 165kDa |
Observed MW: | 165kDa |