Carbamoyl Phosphate Synthase 1, Mitochondrial (CPS1) Antibody

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.

Target	Carbamoyl Phosphate Synthase 1, Mitochondrial (CPS1)
Clonality	Polyclonal
Reactivity	Human
Tested Applications	ELISA, WB
Host	Rabbit
Recommended dilutions	WB: 1/1000. Optimal dilutions/concentrations should be determined by the end user.
Conjugation	Unconjugated
Immunogen	KLH-conjugated synthetic peptide between 262-291 amino acids from the N-terminal region of human CPS1.
Isotype	IgG
Form	Liquid
Purification	Purified through a protein A column, followed by peptide affinity purification.
Storage	Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.
UniProt Primary AC	P31327 (UniProt, ExPASy)
UniProt Secondary AC	B7Z818, J3KQL0, O43774, Q53TL5, Q59HF8, Q7Z5I5
UniProt Entry Name	CPSM_HUMAN
Gene Symbol	CPS1
KEGG	hsa:1373
String	9606.ENSP00000402608
Molecular Weight	Calculated MW: 165 kDa
Buffer	PBS containing 0.09% sodium azide.
Availability	Shipped within 5-10 working days.
Note	This product is for research use only.

Research Articles on Carbamoyl Phosphate Synthase 1, Mitochondrial (CPS1)

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