Application
Immunoperoxidase staining of formalin fixed, paraffin-embedded human breast tissue showing nuclear staining of glandular cells.Application
Western blot analysis of Dyskerin expression in non-transfected: (A) and human Dyskerin transfected: (B) 293T whole cell lysates.Application
Immunofluorescence staining of formalin-fixed HeLa cells showing nucleolar localization. Kindly provided by Dr. Nobuaki Kikyo, Stem Cell Institute, University of Minnesota.Application
Western blot analysis of Dyskerin expression in HeLa (A) and MCF7 (B) nuclear extracts.| Product Name | Dyskerin Antibody (H-300) |
|---|---|
| Antibody Type | Primary Antibodies |
| Antigen Alias | epitope corresponding to amino acids 171-470 mapping within an internal region of Dyskerin of human origin |
| Modification Notes | 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 (1). GAR1, NHP2 and dyskerin localize to the dense fibrillar component of the nucleolus and in nuclear Cajal bodies (2). The dyskerin gene maps to chromosome Xq28 (3). Missense mutations in the dyskerin gene interfere with normal nuclear localization of dyskerin and cause Dyskeratosis congenita (DKC) (4). 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 (5,6). The NHP2 gene maps to chromosome 5q35.3 and encodes a 155-amino acid protein (2,7). |
| Clonality | Polyclonal |
|---|---|
| Isotype | IgG |
| Host Species | Rabbit |
| Tested Applications | WBIPIFIHCELISAGSChIP |
| recommended for detection of Dyskerin of mouse, rat and human origin by WB, IP, IF, IHC(P) and ELISA: |
|
| Species Reactivity | HumanMouseRat |
| Concentration | 1mg/ml |
| Purification | Affinity purified |
| Alternative Names | epitope corresponding to amino acids 171-470 mapping within an internal region of Dyskerin of human origin |
|---|---|
| Tissue Specificity | epitope corresponding to amino acids 171-470 mapping within an internal region of Dyskerin of human origin |
| Entrez Gene | 1736 |
|---|
Application
Immunoperoxidase staining of formalin fixed, paraffin-embedded human breast tissue showing nuclear staining of glandular cells.
Application
Western blot analysis of Dyskerin expression in non-transfected: (A) and human Dyskerin transfected: (B) 293T whole cell lysates.
Application
Immunofluorescence staining of formalin-fixed HeLa cells showing nucleolar localization. Kindly provided by Dr. Nobuaki Kikyo, Stem Cell Institute, University of Minnesota.
Application
Western blot analysis of Dyskerin expression in HeLa (A) and MCF7 (B) nuclear extracts.| Application Notes | recommended for detection of Dyskerin of mouse, rat and human origin by WB, IP, IF, IHC(P) and ELISA: |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles. |
| Storage Buffer | phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. |
