GCH1 Antibody

Catalog Number:

36919

Amount:

100μg

Price:

$319

Swiss-Prot No:

Swiss-Prot#: P30793
NCBI Gene ID: 2643
Gene Accssion: NP_000152

Form of Antibody:

Rabbit IgG in pH7.4 PBS, 0.05% NaN3, 40% Glycerol.

Storage/Stability:

Immunogen:

Synthetic peptide corresponding to a region derived from internal residues of human GTP cyclohydrolase 1

Purification:

Antigen affinity purification.

Specificity/Sensitivity:

The antibody detects endogenous levels of total GCH1 protein.

Applications:

WB IHC

Background:

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.

References:

appl_detail:

Western blotting: 1:200-1:1000
Immunohistochemistry: 1:5-1:20

Datasheet PDF