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You are here:Home » Antibodies » Abs to Mitochondrial Proteins » Anti -CPS1, NT (CPS1, Carbamoyl-phosphate synthase [ammonia], mitochondrial, Carbamoyl-phosphate synthetase I)

Anti -CPS1, NT (CPS1, Carbamoyl-phosphate synthase [ammonia], mitochondrial,
Carbamoyl-phosphate synthetase I)

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Specifications

Clone Host Grade Applications
Polyclonal Rabbit Affinity Purified E B
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.
Catalog #034177
ApplicationsSuitable for use in Western Blot, ELISA
Recommended DilutionELISA: 1:1,000
Western Blot: 1:100-500
Storage and StabilityMay be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Clone TypePolyclonal
IsotypeIgG
HostRabbit
SourceHuman
ConcentrationAs reported
FormSupplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.
PurityPurified by Protein A affinity chromatography.
ImmunogenCPS1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 261-291 amino acids from the N-terminal region of human CPS1.
SpecificityHuman
Important NoteThis product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological.
Alternate namesCPS1, Carbamoyl-phosphate synthase [ammonia], mitochondrial, Carbamoyl-phosphate synthetase I


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