Anti-CPS1 Antibody Picoband®

SKU: A01320-2

Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human,Mouse,Rat,Monkey

Applications: ELISA,Flow Cytometry,IF,IHC,ICC,WB

Application Details: Western blot, 0.1-0.25 μg/ml, Human, Mouse, Rat, Monkey
Immunohistochemistry(Paraffin-embedded Section), 2-5 μg/ml, Human, Mouse, Rat
Immunocytochemistry/Immunofluorescence, 5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: CPS1

Specificity:

Background: Carbamoyl phosphate synthetase I is a ligase enzyme located in the mitochondria involved in the production of urea. 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.

Immunogen: E.coli-derived human CPS1 recombinant protein (Position: N124-E379).

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Adcock, M. W., Ledbetter, D. H., O'Brien, W. E. Analysis of mammalian carbamyl phosphate synthetase I utilizing cDNA clones from human and rat liver. (Abstract) Fed. Proc. 43: 1726, 1984. 2. Adcock, M. W., O'Brien, W. E. Molecular cloning of cDNA for rat and human carbamyl phosphate synthetase I. J. Biol. Chem. 259: 13471-13476, 1984. 3. Aoshima, T., Kajita, M., Sekido, Y., Kikuchi, S., Yasuda, I., Saheki, T., Watanabe, K., Shimokata, K., Niwa, T. Novel mutations (H337R and 238-362del) in the CPS1 gene cause carbamoyl phosphate synthetase I deficiency. Hum. Hered. 52: 99-101, 2001.

Uniprot ID: P31327

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 165 kDa

Calculated Molecular Weight: 60066 MW

Gene ID: 1373

Protein Name: Carbamoyl-phosphate synthase [ammonia], mitochondrial

Gene Full Name: carbamoyl-phosphate synthase 1

Synonyms: CPS1; CPSase I