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.

Pack Size

100ul, 1ml, 20ul

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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