CPS1 Antibody

Primary Antibodies

Blue Ice

165 kDa

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.

None

This antibody is purified through a protein A column, followed by peptide affinity purification.

Supplied in PBS with 0.09% (W/V) sodium azide.

This CPS1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 262-291 amino acids from the N-terminal region of human CPS1.

Carbamoyl-phosphate synthase [ammonia], mitochondrial

Optimal dilutions/concentrations should be determined by the end user. The information provided is a guideline for product use. This product is for research use only.