GCH1 (Human) Recombinant Protein (P01)
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More Files
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Specification
Product Description
Human GCH1 full-length ORF ( AAH25415.1, 1 a.a. - 250 a.a.) recombinant protein with GST-tag at N-terminal.
Sequence
MEKGPVRAPAEKPRGARCSNGFPERDPPRPGPSRPAEKPPRPEAKSAQPADGWKGERPRSEEDNELNLPNLAAAYSSILSSLGENPQRQGLLKTPWRAASAMQFFTKGYQETISDVLNDAIFDEDHDEMVIVKDIDMFSMCEHHLVPFVGKVHIGYLPNKQVLGLSKLARIVEIYSRRLQVQERLTKQIAVAITEALRPAGVGVVVEATHMCMVMRGVQKMNSKTVTSTMLGVFREDPKTREEFLTLIRS
Host
Wheat Germ (in vitro)
Theoretical MW (kDa)
53.24
Preparation Method
Purification
Glutathione Sepharose 4 Fast Flow
Quality Control Testing
12.5% SDS-PAGE Stained with Coomassie Blue.
Storage Buffer
50 mM Tris-HCI, 10 mM reduced Glutathione, pH=8.0 in the elution buffer.
Storage Instruction
Store at -80°C. Aliquot to avoid repeated freezing and thawing.
Note
Best use within three months from the date of receipt of this protein.
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Applications
Enzyme-linked Immunoabsorbent Assay
Western Blot (Recombinant protein)
Antibody Production
Protein Array
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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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Interactome
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Pathway
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Disease
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Publication Reference
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Regulation of Tetrahydrobiopterin Biosynthesis by Shear Stress.
Widder JD, Chen W, Li L, Dikalov S, Thony B, Hatakeyama K, Harrison DG.
Circulation Research 2007 Aug; 101(8):830.
Application:Enzyme, Human, Human aortic endothelial cells (HAECs).
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Regulation of Tetrahydrobiopterin Biosynthesis by Shear Stress.
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