GTP cyclohydrolase 1 (GCH1) Mouse Monoclonal Antibody (Biotin conjugated) [Clone ID: OTI5A1]

Specifications

Product Data
Clone Name	OTI5A1
Applications	IHC, WB
Recommend Dilution	WB 1:2000, IHC 1:500
Reactivity	Human, Mouse, Rat
Host	Mouse
Clonality	Monoclonal
Immunogen	Full length human recombinant protein of human GCH1 (NP_001019195) produced in E.coli.
Formulation	PBS (pH 7.3) containing 1% BSA, 50% glycerol and 0.02% sodium azide.
Concentration	0.5 mg/ml
Purification	Purified from mouse ascites fluids or tissue culture supernatant by affinity chromatography (protein A/G)
Conjugation	Biotin
Storage Condition	Store at -20°C as received.
Predicted Protein Size	27.7 kDa
Gene Name	GTP cyclohydrolase 1
Database Link	NP_001019195 Entrez Gene 14528 MouseEntrez Gene 29244 RatEntrez Gene 2643 Human UniProt ID P30793
Background	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]
Synonyms	DYT5; DYT5a; DYT14; GCH; GTP-CH-1; GTPCH1; HPABH4B
Reference Data
Protein Families	Druggable Genome
Protein Pathways	Folate biosynthesis, Metabolic pathways

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