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ERCC6/CSB Rabbit anti-Human, Polyclonal, Proteintech
Rabbit Polyclonal Antibody
Marque: Proteintech 24291-1-AP-20UL
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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.Spécification
| ERCC6/CSB | |
| Polyclonal | |
| Unconjugated | |
| ERCC6 | |
| ARMD5, ATP dependent helicase ERCC6, CKN2, Cockayne syndrome protein CSB, COFS, COFS1, CSB, ERCC6, RAD26 | |
| Rabbit | |
| Antigen Affinity Chromatography | |
| RUO | |
| 2074 | |
| -20°C | |
| Liquid |
| Western Blot, Immunoprecipitation | |
| 0.59 mg/mL | |
| PBS with 50% glycerol and 0.1% sodium azide; pH 7.3 | |
| Q03468 | |
| ERCC6 | |
| ERCC6/CSB Fusion Protein Ag18009 | |
| 20 μL | |
| Primary | |
| Human | |
| Antibody | |
| IgG |
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