FluoroFinder

	Supplier	Biorbyt
	Catalog #:	orb388005-100ug (View supplier product page)
	Size	100 μg
	Price	$412.00
	Antigen	CPS1
	Clone	CPS1/1022
	Host	Mouse
	Isotype	IgG1
	Conjugate	Unconjugated
	Target Species	Canine, Human
	Applications	IHC-P, IF, ICC
	Description	Mouse monoclonal antibody to CPS1

	CPS1	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]