ERCC6/CSB Rabbit anti-Human, Polyclonal, Proteintech

Rabbit Polyclonal Antibody

Catalog Number	Mfr. No.	Quantity
50-173-4281	Proteintech Group Inc 242911AP150UL	150 μL

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
• Applications: Immunoprecipitation, Western Blot
• Conjugate: Unconjugated
• Host Species: Rabbit
• Target Species: Human
• Gene Accession No.: Q03468
• Gene ID (Entrez): 2074
• Immunogen: ERCC6/CSB Fusion Protein Ag18009
• Primary or Secondary: Primary
• Content And Storage: -20°C
• Gene: ERCC6

• Concentration: 0.59 mg/mL
• Classification: Polyclonal
• Form: Liquid
• Regulatory Status: RUO
• Formulation: PBS with 50% glycerol and 0.1% sodium azide; pH 7.3
• Gene Alias: ARMD5, ATP dependent helicase ERCC6, CKN2, Cockayne syndrome protein CSB, COFS, COFS1, CSB, ERCC6, RAD26
• Gene Symbols: ERCC6
• Isotype: IgG
• Purification Method: Antigen Affinity Chromatography
• Product Type: Antibody