Product Name	GCH1
Chinese Name	三磷酸鸟苷环水解酶抗体
Alias	GTP-CH-1; DYT 5; DYT5; GCH 1; GCH; GTP CH 1; GTP CH I; GTP cyclohydrolase 1 (dopa responsive dystonia); GTP cyclohydrolase 1; GTP cyclohydrolase I; GTPCH 1; GTPCH1; Guanosine 5' triphosphate cyclohydrolase I; DYT14; DYT5a; GCH1_HUMAN; HPABH4B.
Research Area	immunology
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Human,  (predicted: Mouse, Rat, Chicken, Dog, Cow, Horse, Sheep, )
Applications	WB=1:500-2000 ELISA=1:5000-10000  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	28kDa
Cellular localization	The nucleus cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human GCH1: 101-200/250
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	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] SWISS: P30793 Gene ID: 2643 Database links: Entrez Gene: 2643 Human Entrez Gene: 14528 Mouse Entrez Gene: 100286831 Rabbit Entrez Gene: 29244 Rat Omim: 600225 Human SwissProt: P30793 Human SwissProt: Q05915 Mouse SwissProt: P51596 Rabbit SwissProt: P22288 Rat Unigene: 624900 Human Unigene: 86724 Human Unigene: 10651 Mouse Unigene: 28195 Rat
Product Picture	Sample: Lane 1: Recombinant human GCH1 protein, C-His(SL42069P) Primary: Anti-GCH1 (SL25514R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 28 kDa Observed band size: 30 kDa

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