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Gel: 12%SDS-PAGE, Lysate: 60 μg, Lane: Human fetal liver tissue, Primary antibody: P10938(GCH1 Antibody) at dilution 1/200, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 15 seconds

The image is immunohistochemistry of paraffin-embedded Human breast cancer tissue using P10938(GCH1 Antibody) at dilution 1/15. (Original magnification: ×200)

Rabbit Polyclonal Antibody to GCH1

货号：
P10938
别名：
GCH, DYT5, DYT14, DYT5a, GTPCH1, HPABH4B, GTP-CH-1
产品上架时间：
2024-04-06
应用：
WB,IHC
反应种属：
Human
抗体类型：
Primary antibody
Swissprot：
P30793
规格：

100μl
50μl
20μl
数量：

-

+
说明书：

目录价¥1980

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Rabbit Polyclonal Antibody to GCH1

Description
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.

Description

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.

Specification
Aliases	GCH, DYT5, DYT14, DYT5a, GTPCH1, HPABH4B, GTP-CH-1
Swissprot	P30793
WB Predicted band size	28 kDa
Host/Isotype	Rabbit IgG
Antibody Type	Primary antibody
Storage	Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species Reactivity	Human
Immunogen	Synthetic peptide of human GCH1
Formulation	Purified antibody in PBS with 0.05% sodium azide and 50% glycerol.