DKC1 Rabbit Polyclonal Antibody

SKU AP06643PU-N

DKC1 rabbit polyclonal antibody, Aff - Purified

Product Data

• Application: IF, IHC, WB
• Recommended Dilution: Western blot: 1/500-1/1000.; Immunofluorescence: 1/50-1/200.; Immunohistochemistry on Paraffin Sections: 1/50-1/200.
• Reactivity: Human, Mouse, Rat
• Antibody Host: Rabbit
• Clonality: Polyclonal
• Immunogen: Synthetic peptide, corresponding to amino acids 171-220 of Human Dyskerin.
• Specificity: The antibody detects endogenous levels of Dyskerin protein.; (region surrounding Lys203)
• Buffer: Phosphate buffered saline (PBS), pH 7.2.; State: Aff - Purified; State: Liquid purified Ig fraction (> 95% by SDS-PAGE); Preservative: 0.05% Sodium Azide
• Concentration: 1.0 mg/ml
• Purification: Affinity Chromatography using epitope-specific immunogen
• Conjugation: Unconjugated
• Storage: Store undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer.; Avoid repeated freezing and thawing.
• Stability: Shelf life: one year from despatch.
• Shipping: Blue Ice
• Predicted Protein Size: ~ 57 kDa
• Gene Name: dyskerin pseudouridine synthase 1
• Database Link:
  O60832
• Background: Dyskerin (NAP57) associates with the chaperone protein Nopp140 and forms a small ribonucleoprotein particle with GAR1 (NOLA1), NHP2 (NOLA2) and Nop10 for the isomerization of uridine to pseudouridine. GAR1, NHP2 and Dyskerin localize to the dense fibrillar component of the nucleolus and in nuclear Cajal bodies. The Dyskerin gene maps to chromosome Xq28. Missense mutations in the Dyskerin gene interfere with normal nuclear localization of Dyskerin and cause Dyskeratosis congenita (DKC). DKC is a rare, X-linked bone marrow disorder characterized by cutaneous hyperpigmentation, dystrophy of the nails, atrophy of the testicles and leukoplakia of the oral mucosa. The GAR1 gene maps to chromosome 4q25 and encodes a 28 kDa protein. The NHP2 gene maps to chromosome 5q35.3 and encodes a 155 amino acid protein.
• Synonyms: Dyskerin, Nucleolar protein NAP57, CBF5 homolog

Reference Data