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Overview
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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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- Properties
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Overview
Immunofluorescence analysis of HeLa cells using CPS1 Polyclonal Antibody (STJ110382) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.
Western blot analysis of extracts of various cell lines, using CPS1 antibody (STJ110382) at 1:1000 dilution.Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) at 1:10000 dilution.Lysates/proteins: 25ug per lane.Blocking buffer: 3% nonfat dry milk in TBST.Detection: ECL Basic Kit.Exposure time: 1s.
Anti-CPS1 antibody
Anti-CPS1 antibody
Catalog Number:
A001017216STJ
Mfr. No.:
STJ110382
Price:
$297
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