Anti-CPS1 Mouse Monoclonal Antibody
Product Information
Downloads & Supports
Target
-
CPS1
Host
Storage
-
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Clonality
Clone Number
-
ZN281
Isotype
-
IgG1
Reactivity
Application
Application Details
-
IHC: 1/50-1/100
Immunogen
-
Human recombinant protein fragment of human CPS1 (NP_001116105) produced in E.coli.
Molecular Weight
-
-
Formulation
-
PBS (PH 7.3) containing 1% BSA, 50% glycerol and 0.02% sodium azide.
Form
-
Liquid
Gene ID
-
1373
Uniprot ID
-
P31327
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].
Research Area
-
Tags & Cell Markers
Synonyms
-
Cps1; CPSase 1; CPSase I; CPSASE1