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Dyskerin pAb

Dyskerin pAb

Cat no: BS6586

Supplier: Bioworld Technology, Inc.
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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.
Catalogue number: BS6586
Reactivities: Human, Mouse, Rat
Hosts: Rabbit
Applications: Immunohistochemistry, Western Blot
Size: 100ug/100ul
Swiss prot: O60832
Dilutions: WB: 1:500~1:2000 IHC: 1:50~1:200
Form: liquid
Storage buffer: 1 mg/ml in Phosphate buffered saline (PBS) with 0.05% sodium azide, approx. pH 7.2.
Concentration: 1ug/ul
Antigen: Recombinant full length Human Dyskerin.
Species: Dyskerin pAb detects endogenous levels of Dyskerin protein.
Molecular weight: ~ 57 kDa
Purity: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Storage temp: Store at 4 degrees C short term. Aliquot and store at -20 degrees C long term. Avoid freeze-thaw cycles.
Alternative names: H/ACA ribonucleoprotein complex subunit 4; CBF5 homolog; Dyskerin; Nopp140-associated protein of 57 kDa; Nucleolar protein NAP57; Nucleolar protein family A member 4; snoRNP protein DKC1; DKC1; NOLA4
Additional info: For research use only, not for use in diagnostic procedure.

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