CPS1 antibody (clone 8H8), anti-human
€435.00
In stock
SKU
AC-AT1608a
Catalog Number: AC-AT1608a
Size: 100 µg
Isotype: mouse IgG1 Kappa
Applications: WB, IHC, E
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Size: 100 µg
Isotype: mouse IgG1 Kappa
Applications: WB, IHC, E
Request Information AC-AT1608a">Request Information
Background:
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.
Other Names:
Carbamoyl-phosphate synthase [ammonia], mitochondrial, Carbamoyl-phosphate synthetase I, CPSase I, CPS1
Antigen Type:
Recombinant Protein
Gene Name: CPS1
Gene ID: 1373
NCBI Accession: NP_001116105.1;NP_001116106.1;NP_001866.2
Primary Accession: P31327
Other Accession: NM_001875
Format: Clear, colorless solution in phosphate buffered saline, pH 7.2 .
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.
Other Names:
Carbamoyl-phosphate synthase [ammonia], mitochondrial, Carbamoyl-phosphate synthetase I, CPSase I, CPS1
Antigen Type:
Recombinant Protein
Gene Name: CPS1
Gene ID: 1373
NCBI Accession: NP_001116105.1;NP_001116106.1;NP_001866.2
Primary Accession: P31327
Other Accession: NM_001875
Format: Clear, colorless solution in phosphate buffered saline, pH 7.2 .
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