GCH1 monoclonal antibody (M02), clone 2C4
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More Files
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Specification
Product Description
Mouse monoclonal antibody raised against a full length recombinant GCH1.
Immunogen
GCH1 (AAH25415.1, 1 a.a. ~ 250 a.a) full-length recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
MEKGPVRAPAEKPRGARCSNGFPERDPPRPGPSRPAEKPPRPEAKSAQPADGWKGERPRSEEDNELNLPNLAAAYSSILSSLGENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIVEIYSRRLQVQERLTKQIAVAITEALRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLTLIRS
Host
Mouse
Reactivity
Human
Isotype
IgG1 Kappa
Quality Control Testing
Antibody Reactive Against Recombinant Protein.
Storage Buffer
In 1x PBS, pH 7.4
Storage Instruction
Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing.
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Applications
Sandwich ELISA (Recombinant protein)
Detection limit for recombinant GST tagged GCH1 is approximately 0.1ng/ml as a capture antibody.ELISA
Immunofluorescence
Immunofluorescence of monoclonal antibody to GCH1 on HeLa cell . [antibody concentration 10 ug/ml] -
Gene Info — GCH1
Entrez GeneID
2643GeneBank Accession#
BC025415Protein Accession#
AAH25415.1Gene Name
GCH1
Gene Alias
DYT14, DYT5, GCH, GTP-CH-1, GTPCH1
Gene Description
GTP cyclohydrolase 1
Gene Ontology
HyperlinkGene 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
Other Designations
guanosine 5'-triphosphate cyclohydrolase I
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Disease
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