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ERCC6/CSB Rabbit anti-Human, Polyclonal, Proteintech

Product Code. 16852765 Shop All Proteintech Products
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150 μL
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16852765 150 μL 150µL
16862765 20 μL 20µL
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Product Code. 16852765 Supplier Proteintech Supplier No. 242911AP150UL

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Description

Rabbit Polyclonal Antibody

This gene encodes a member of the GTP cyclohydrolase family. The encoded protein is the first and rate-limiting enzyme in tetrahydrobiopterin 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 maligt 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.

Specifications

Antigen ERCC6/CSB
Applications Immunoprecipitation, Western Blot
Classification Polyclonal
Concentration 0.59 mg/mL
Conjugate Unconjugated
Formulation PBS with 50% glycerol and 0.1% sodium azide; pH 7.3
Gene ERCC6
Gene Accession No. Q03468
Gene Alias ARMD5, ATP dependent helicase ERCC6, CKN2, Cockayne syndrome protein CSB, COFS, COFS1, CSB, ERCC6, RAD26
Gene Symbols ERCC6
Host Species Rabbit
Immunogen ERCC6/CSB Fusion Protein Ag18009
Purification Method Antigen Affinity Chromatography
Quantity 150 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 2074
Target Species Human
Content And Storage -20°C
Product Type Antibody
Form Liquid
Isotype IgG
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