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.
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Pack Size | 100ul, 1ml, 20ul |
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Applications | ELISA, IF/ICC, IHC-P, WB |
Host Species | Rabbit |
Ig Isotype | IgG |
Antibody Type | Monoclonal Antibody |
Organism Species | Human |
Product Synonyms | CPS1 |
Observed Mol Wt | 165kDa |
Alternative Names | PHN; GATD6; CPSASE1; CPS1 |
Immunogen (Antigen) | synthetic peptide |
Format | Liquid |
Buffer Formulation | 0.05% BSA, 50% glycerol, PBS with 0.02% sodium azide, pH7.3 |
Reactivity | Human, Mouse, Rat |
Uniprot ID | 1373 |
Gene ID | P31327 |
Potency (Clone Number) | ARC0929 |
Purification | Antigen-specific affinity chromatography followed by Protein A affinity chromatography |
Usage | For Research Use Only. Not for diagnostics or human use. |
Shelf Life | 12 months at time of shipping |
Shipping | Shipped in Dry Ice at -20 Degree Celsius |
Storage | Store at -20 Degree Celsius. It is recommended to aliquot and store to avoid repeated freeze-thaw as it affects the stability of the antibody. |
Research Areas | Cancer, Cell Biology, Immunology |
KD/KO Validated | KD Validated |
Disclaimer | The data indicated herein are as indicated and validated in our laboratory. These reagents are for research use only and not for in-vitro diagnostics or human use. |
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