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Product Details
PA5-117888
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human
Host/Isotype
Rabbit
/ IgG
Class
Polyclonal
Type
Antibody
Immunogen
E. coli-derived Human CPS1 fragment
Conjugate
Form
Liquid
Purification
Antigen affinity chromatography, Protein A
Storage buffer
PBS, pH 7
Contains
0.03% ProClin 300
Storage conditions
-20° C, Avoid Freeze/Thaw Cycles
Shipping conditions
Wet ice
RRID
AB_2902496
Target Information
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: carbamoyl-phosphate synthase (ammonia); carbamoyl-phosphate synthase 1, mitochondrial; Carbamoyl-phosphate synthase [ammonia], mitochondrial; Carbamoyl-phosphate synthetase I; carbamoylphosphate synthetase I; CPSase I
Gene Aliases: CPS1; CPSASE1; PHN
UniProt ID: (Human) P31327
Entrez Gene ID: (Human) 1373
aspartate carbamoyltransferase activity
carbamoyl-phosphate synthase (ammonia) activity
carbamoyl-phosphate synthase (glutamine-hydrolyzing) activity
dihydroorotase activity
endopeptidase activity
calcium ion binding
protein binding
ATP binding
phospholipid binding
glutamate binding
protein complex binding
modified amino acid binding
ligase
urea cycle
glycogen catabolic process
'de novo' pyrimidine nucleobase biosynthetic process
proteolysis
arginine biosynthetic process
glutamine metabolic process
midgut development
response to toxic substance
response to zinc ion
response to amine
citrulline biosynthetic process
triglyceride catabolic process
response to food
response to lipopolysaccharide
response to drug
response to starvation
response to amino acid
cellular response to fibroblast growth factor stimulus
positive regulation of vasodilation
nitric oxide metabolic process
homocysteine metabolic process
anion homeostasis
response to growth hormone
hepatocyte differentiation
carbamoyl phosphate biosynthetic process
cellular response to cAMP
cellular response to glucagon stimulus
cellular response to oleic acid
response to dexamethasone
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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