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产品信息
H00002643-M02
产品规格
种属反应
Human
宿主/亚型
Mouse
/ IgG1, kappa
分类
Monoclonal
类型
Antibody
克隆号
2C4
抗原
GCH1 (AAH25415.1, 1 a.a. approximately 250 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
偶联物
形式
Liquid
浓度
See Label
纯化类型
Affinity chromatography
保存液
PBS, pH 7.4
内含物
no preservative
保存条件
-20° C, Avoid Freeze/Thaw Cycles
运输条件
Ambient (domestic); Wet ice (international)
产品详细信息
Sequence of this protein is as follows: MEKGPVRAPA EKPRGARCSN GFPERDPPRP GPSRPAEKPP RPEAKSAQPA DGWKGERPRS EEDNELNLPN LAAAYSSILS SLGENPQRQG LLKTPWRAAS AMQFFTKGYQ ETISDVLNDA IFDEDHDEMV IVKDIDMFSM CEHHLVPFVG KVHIGYLPNK QVLGLSKLAR IVEIYSRRLQ VQERLTKQIA VAITEALRPA GVGVVVEATH MCMVMRGVQK MNSKTVTSTM LGVFREDPKT REEFLTLIRS
靶标信息
This gene encodes a member of the GTP cyclohydrolase family. The encoded protein is the first and rate-limiting enzyme in tetrahydrobiopterin biosynthesis, catalyzing the conversion of GTP into 7,8-dihydroneopterin triphosphate. BH4 is an essential cofactor required by aromatic amino acid hydroxylases as well as nitric oxide synthases. Mutations in this gene are associated with malignant hyperphenylalaninemia and dopa-responsive dystonia. Several alternatively spliced transcript variants encoding different isoforms have been described; however, not all variants give rise to a functional enzyme.
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: dystonia 14; GTP cyclohydrolase 1; GTP cyclohydrolase I; GTP-CH-I; guanosine 5'-triphosphate cyclohydrolase I
基因别名: DYT14; DYT5; DYT5a; GCH; GCH1; GTP-CH-1; GTPCH1; HPABH4B
UniProt ID: (Human) P30793
Entrez Gene ID: (Human) 2643
tetrahydrobiopterin biosynthetic process
nitric oxide biosynthetic process
regulation of blood pressure
regulation of lung blood pressure
response to lipopolysaccharide
response to interferon-gamma
response to tumor necrosis factor
7,8-dihydroneopterin 3'-triphosphate biosynthetic process
vasodilation
dopamine biosynthetic process
pteridine-containing compound biosynthetic process
negative regulation of blood pressure
tetrahydrofolate biosynthetic process
response to pain
neuromuscular process controlling posture
regulation of nitric-oxide synthase activity
positive regulation of nitric-oxide synthase activity
protein homooligomerization
protein heterooligomerization
regulation of removal of superoxide radicals
