-
Product Name
GCH1 Rabbit pAb
-
Gene ID
2643
-
SwissProt ID
P30793
-
Gene Name
GCH1
-
Alternative Names
GCH; DYT5; DYT14; DYT5a; GTPCH1; HPABH4B; GTP-CH-1
-
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.
-
Research Field
Neuroscience
-
Product Categories
Primary antibody
-
Host
Rabbit
-
Reactivity
Human
-
Application
WB,IHC-P
-
Dilution Ratio
WB: 1/500-1/1000 IHC: 1/50-1/100
-
Molecular Weight
Calculated MW: 28 kDa; Observed MW: 28 kDa
-
Clonality
Polyclonal Antibody
-
Clonality No.
-
-
Isotype
IgG
-
Immunogen
Synthetic peptide of human GCH1
-
Purification
Affinity Purified
-
Conjugation
Unconjugated
-
Modification
Unmodified
-
Form
Liquid
-
Buffer System
pH 7.4 PBS, 0.05% NaN3, 40% Glycerol
-
Storage
Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.