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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Western blotting analysis using Carbamoyl-phosphate synthase 1 antibody . Total cell lysates (30 μg) from various cell lines were loaded and separated by SDS-PAGE. The blot was incubated with Carbamoyl-phosphate synthase 1 antibody and HRP-conjugated goat anti-rabbit secondary antibody respectively. Image was developed using FeQ™ ECL Substrate Kit .

Western blotting analysis using carbamoyl-phosphate synthase 1 antibody . Carbamoyl-phosphate synthase 1 expression in wild type (WT) and carbamoyl-phosphate synthase 1 (CPS1) shRNA knockdown (KD) HeLa cells with 30 μg of total cell lysates. Hsp90 α serves as a loading control. The blot was incubated with carbamoyl-phosphate synthase 1 antibody and HRP-conjugated goat anti-rabbit secondary antibody respectively. Image was developed using FeQ™ ECL Substrate Kit .

Flow cytometric analysis of Carbamoyl-phosphate synthase 1 expression in HepG2 cells using Carbamoyl-phosphate synthase 1 antibody . Green, isotype control; red, Carbamoyl-phosphate synthase 1.

Validation of Carbamoyl-phosphate synthase 1 knockdown using flow cytometry. Wild-type(WT, Blue) and knockdown(KD, Green) HeLa cells were stained with Carbamoyl-phosphate synthase 1 antibody and analyzed using BD flow cytometer.

Immunocytochemical staining of HepG2 cells with Carbamoyl-phosphate synthase 1 antibody . Nuclei were stained blue with DAPI; Carbamoyl-phosphate synthase 1 was stained magenta with Alexa Fluor® 647. Images were taken using Leica stellaris 5. Protein abundance based on laser Intensity and smart gain: High. Scale bar, 20 μm.

Immunocytochemical staining of Hela cells using Carbamoyl-phosphate synthase 1 antibody , Top panel: wild-type (WT); Bottom panal: Carbamoyl-phosphate synthase 1 shRNA knockdown (KD). Nuclei were stained blue with DAPI; Carbamoyl-phosphate synthase 1 was stained magenta with Alexa Fluor® 647. Scale bar, 20 μm.

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