bsm-54314R

DATASHEET

Host: Rabbit

Target Protein: CPS1

Clonality: Monoclonal

Isotype: IgG

Swiss Prot: P31327

Source: Recombinant protein within human CPS1 aa 50-200

Purification: Purified by Protein A.

Storage Buffer: 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Storage: Store at 4°C for up to 2 weeks. For long term storage, store at -20°C in small aliquots to prevent freeze-thaw cycles.

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.[provided by RefSeq, May 2010]

Size: 100ul

Concentration: 1ug/ul

Cross Reactive Species: Human
Mouse
Rat

For research use only. Not intended for diagnostic or therapeutic use.