CPS1 Antibody

Catalog Number:

46982

Amount:

100μg

Price:

$319

Swiss-Prot No:

Swiss-Prot#:P31327
NCBI Gene ID:1373
Gene Accssion:NP_001866

Form of Antibody:

Rabbit IgG in pH7.4 PBS, 0.05% NaN3, 40% Glycerol.

Storage/Stability:

Immunogen:

Synthetic peptide of human CPS1

Purification:

Antigen affinity purification

Specificity/Sensitivity:

The antibody detects endogenous levels of total CPS1 protein.

Applications:

IHC

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.

References:

appl_detail:

Immunofluorescence:1: 25-100

Datasheet PDF