ERCC6/CSB Rabbit anti-Human, Polyclonal, Proteintech

Rabbit Polyclonal Antibody

Supplier:  Proteintech Group Inc 242911AP150UL

Catalog No. 50-173-4281

Description

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
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: ERCC6
• Gene Alias: ARMD5, ATP dependent helicase ERCC6, CKN2, Cockayne syndrome protein CSB, COFS, COFS1, CSB, ERCC6, RAD26
• Host Species: Rabbit
• Purification Method: Antigen Affinity Chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 2074
• Content And Storage: -20°C
• Form: Liquid

• Applications: Immunoprecipitation, Western Blot
• Concentration: 0.59 mg/mL
• Formulation: PBS with 50% glycerol and 0.1% sodium azide; pH 7.3
• Gene Accession No.: Q03468
• Gene Symbols: ERCC6
• Immunogen: ERCC6/CSB Fusion Protein Ag18009
• Quantity: 150 μL
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG