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产品信息
PA5-117888
应用
建议稀释比
已发表文章
产品规格
种属反应
Human
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
E. coli-derived Human CPS1 fragment
偶联物
形式
Liquid
纯化类型
Antigen affinity chromatography, Protein A
保存液
PBS, pH 7
内含物
0.03% ProClin 300
保存条件
-20° C, Avoid Freeze/Thaw Cycles
运输条件
Wet ice
RRID
AB_2902496
靶标信息
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.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
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生物信息学
蛋白别名: carbamoyl-phosphate synthase (ammonia); carbamoyl-phosphate synthase 1, mitochondrial; Carbamoyl-phosphate synthase [ammonia], mitochondrial; Carbamoyl-phosphate synthetase I; carbamoylphosphate synthetase I; CPSase I
基因别名: 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
