BS6586
antibody from Bioworld Technology, Inc
Targeting: DKC1
Cbf5, DKC, dyskerin, NAP57, NOLA4, XAP101
Antibody data
- Antibody Data
- Antigen structure
- References [0]
- Comments [0]
- Validations
- Western blot [1]
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- Product number
- BS6586 - Provider product page
- Provider
- Bioworld Technology, Inc
- Product name
- Dyskerin polyclonal antibody
- Antibody type
- Polyclonal
- Antigen
- Recombinant full length Human Dyskerin.
- Description
- 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. The NHP2 gene maps to chromosome 5q35.3 and encodes a 155-amino acid protein.
- Reactivity
- Human, Mouse, Rat
- Host
- Rabbit
- Isotype
- IgG
- Vial size
- 100ul
- Concentration
- 1 mg/ml
- Storage
- Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
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Supportive validation
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- Bioworld Technology, Inc (provider)
- Main image
- Experimental details
- Western blot analysis of extracts of various cell lines, using Dyskerin antibody.