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Description
The antibody is specific to ERCC6.
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
Specifications
| Antigen | ERCC6/CSB |
| Applications | Western Blot |
| Classification | Polyclonal |
| Concentration | 0.17 mg/mL |
| Conjugate | Unconjugated |
| Formulation | PBS with 50% glycerol and 0.02% 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 |
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Product Title
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