catalog number :
MBS421787
products full name :
Goat anti-GCH1 Antibody
products short name :
GCH1
products name syn :
GCH1; GTP cyclohydrolase 1 (dopa-responsive dystonia); DYT5; GCH; GTP-CH-1; GTPCH1; GTP cyclohydrolase 1; guanosine 5'-triphosphate cyclohydrolase I; GCH1 antibody; GTP cyclohydrolase 1 (dopa-responsive dystonia) antibody; DYT5 antibody; GCH antibody; GTP-CH-1 antibody; GTPCH1 antibody; GTP cyclohydrolase 1 antibody; guanosine 5 1-triphosphate cyclohydrolase I antibody
other names :
GTP cyclohydrolase 1 isoform 1; GTP cyclohydrolase 1; GTP cyclohydrolase 1; GTP cyclohydrolase 1; GTP cyclohydrolase I; GTP-CH-I
products gene name :
GCH1
other gene names :
GCH1; GCH1; GCH; DYT5; DYT14; DYT5a; GTPCH1; HPABH4B; GTP-CH-1; DYT5; GCH; GTP-CH-I
uniprot entry name :
GCH1_HUMAN
reactivity :
Tested: Human; Expected from sequence similarity: Human, Mouse, Rat
purity :
Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide
form :
Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.
concentration :
100ug specific antibody in 200ul
storage stability :
Aliquot and store at -20 degree C. Minimize freezing and thawing.
tested application :
Peptide ELISA (EIA), Western Blot (WB)
app notes :
Peptide ELISA: Antibody detection limit dilution 1: 16000. Western Blot: Approx.37kDa band observed in Human Tonsil, Lymph Node and Thymus lysates. Customer's experiments gave identical results in lysates of cell line HUVEC with antibodies raised against different parts of the same protein. (calculated MW of 27.9kDa according to NP_000152.1). Recommended concentration: 0.5-1.5ug/ml.
other info1 :
Immunogen: Peptide with sequence C-GKVHIGYLPNKQ, from the internal region of the protein sequence according to NP_000152.1 ; NP_001019195.1 ; NP_001019241.1 ; NP_001019242.1. Epitope: Internal region
other info2 :
Note: This antibody is expected to recognize all four isoforms (NP_000152.1 ; NP_001019195.1 ; NP_001019241.1 ; NP_001019242.1)
ncbi acc num :
NP_000152.1
ncbi gb acc num :
NM_000161.2
ncbi mol weight :
25,775 Da
ncbi pathways :
Folate Biosynthesis Pathway (83018); Folate Biosynthesis Pathway (404); Metabolic Pathways (132956); Metabolism Pathway (1269956); Metabolism Of Nitric Oxide Pathway (1270115); Tetrahydrobiopterin (BH4) Synthesis, Recycling, Salvage And Regulation Pathway (1270120); ENOS Activation And Regulation Pathway (1270116); Tetrahydrobiopterin Biosynthesis I Pathway (138454); Tetrahydrobiopterin Biosynthesis II Pathway (138455); Tetrahydrobiopterin De Novo Biosynthesis Pathway (142229)
ncbi summary :
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. [provided by RefSeq, Jul 2008]