GCH1 purified MaxPab rabbit polyclonal antibody (D01P)
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More Files
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Specification
Product Description
Rabbit polyclonal antibody raised against a full-length human GCH1 protein.
Immunogen
GCH1 (NP_000152.1, 1 a.a. ~ 250 a.a) full-length human protein.
Sequence
MEKGPVRAPAEKPRGARCSNGFPERDPPRPGPSRPAEKPPRPEAKSAQPADGWKGERPRSEEDNELNLPNLAAAYSSILSSLGENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIVEIYSRRLQVQERLTKQIAVAITEALRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLTLIRS
Host
Rabbit
Reactivity
Human
Quality Control Testing
Antibody reactive against mammalian transfected lysate.
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
Western Blot (Transfected lysate)
Western Blot analysis of GCH1 expression in transfected 293T cell line (H00002643-T01) by GCH1 MaxPab polyclonal antibody.
Lane 1: GCH1 transfected lysate(27.90 KDa).
Lane 2: Non-transfected lysate.
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Gene Info — GCH1
Entrez GeneID
2643GeneBank Accession#
NM_000161.2Protein Accession#
NP_000152.1Gene 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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