GCH1 Polyclonal Antibody

Rs. 16,500.00
SKU E-AB-15705

Overview

Synonyms dystonia 14,DYT 5,DYT14,DYT5,DYT5a,GCH 1,GCH,Gch1,GCH1,GTP CH 1,GTP CH I,GTP cyclohydrolase 1 (dopa responsive dystonia),GTP cyclohydrolase 1,GTP cyclohydrolase I,GTP-CH-I,GTPCH 1,GTPCH1,Guanosine 5' triphosphate cyclohydrolase I,HPABH4B
Swissprot P30793
Source Rabbit
Reactivity Human
Immunogen Synthetic peptide of human GCH1
Application WB,IHC,ELISA
Recommended dilution WB 1:500-1:2000, IHC 1:5-1:20
Concentration 0.3mg/mL
Clonality Polyclonal

Properties

Cellular localization  
Tissue specificity  
Isotype IgG
Purification Affinity purification
Conjugation Unconjugated
Storage instructions Store at -20℃. Avoid freeze / thaw cycles.
Storage buffer PBS with 0.05% sodium azide, 50% glycerol, PH7.3
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.

 

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