GCH1 Antibody - Cat. #: CSB-PA964562

SKU: CSB-PA964562

Size :50ul

Clone Number:

Aliases:dystonia 14 antibody; DYT 5 antibody; DYT14 antibody; DYT5 antibody; DYT5a antibody; GCH 1 antibody; GCH antibody; Gch1 antibody; GCH1_HUMAN antibody; GTP CH 1 antibody; GTP CH I antibody; GTP cyclohydrolase 1 (dopa responsive dystonia) antibody; GTP cyclohydrolase 1 antibody; GTP cyclohydrolase I antibody; GTP-CH-I antibody; GTPCH 1 antibody; GTPCH1 antibody; Guanosine 5' triphosphate cyclohydrolase I antibody; HPABH4B antibody

Product Type:Polyclonal Antibody

Immunogen Species:Homo sapiens (Human)

UniProt ID:P30793

Immunogen:Synthetic peptide of Human GCH1

Raised in:Rabbit

Species Reactivity:Human

Tested Applications:ELISA, WB, IHC; ELISA:1:1000-1:5000, WB:1:500-1:2000, IHC:1:5-1:20

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.

Clonality:Polyclonal

Isotype:IgG

Purification Method:Antigen affinity purification

Conjugate:Non-conjugated

Buffer:-20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol

Form:Liquid

Stroage:Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.

Target Names:GCH1

Research Areas:Neuroscience?Cancer;Cell biology;Metabolism;Signal transduction