Rabbit Polyclonal Antibody to GCH1

包装: 1G|100G|500G|1KG|100KG

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别名:GCH, DYT5, DYT14, DYT5a, GTPCH1, HPABH4B, GTP-CH-1
应用:WB,IHC
反应种属:Human
规格：50μl/100μl

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
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	pH7.4 PBS, 0.05% NaN3, 40% Glycerol

Application
WB	1/500-1/2000
IHC	1/5-1/20
ELISA	1/1000-1/5000

	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)