深圳欣博盛生物科技有限公司 a1

GCH1 antibody, Internal
货号:GTX88689 规格:100μg 目录价:¥5600
产品详情
* 以下信息仅供参考,详情请以原厂网站为准
产品名称:
GCH1 antibody, Internal
别名:
GTP cyclohydrolase 1 , DYT14 , DYT5 , DYT5a , GCH , GTP-CH-1 , GTPCH1 , HPABH4B
反应种属:
Human
宿主来源:
Goat
实验应用:
WB
靶标/特异性:
This antibody is expected to recognize all four isoforms (NP_000152.1 ; NP_001019195.1 ; NP_001019241.1 ; NP_001019242.1)
同种型:
IgG
免疫原:
Peptide with sequence C-GKVHIGYLPNKQ, from the internal region of the protein sequence according to NP_000152.1 ; NP_001019195.1 ; NP_001019241.1 ; NP_001019242.1 .
克隆性:
Polyclonal
克隆号:
纯化方式:
Purified by ammonium sulphate precipitation followed by antigen affinity chromatography
偶联:
Unconjugated
产品浓度:
0.50 mg/ml (Please refer to the vial label for the specific concentration.)
保存温度:
Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
运输温度:
4°C
预期分子量:
28
产品形式:
Liquid
存储溶液:
TBS, 0.5% BSA, 0.02% Sodium azide.
生产商:
GeneTex
功能与背景:
This gene encodes a member of the GTP cyclohydrolase family. The encoded protein is the first and rate-limiting enzyme in tetrahydrobiopterin (BH4) 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. [provided by RefSeq, Jul 2008]
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