BS6586

antibody from Bioworld Technology, Inc
Targeting: DKC1 Cbf5, DKC, dyskerin, NAP57, NOLA4, XAP101
Provider product page for BS6586
Western blot
Immunocytochemistry
Immunohistochemistry

Antibody data

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.
DKC1 protein structure - BS6586 shown in red.