Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| H | IF, IP, WB | Rb | Affinity Purified | Polyclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | ABE2587 |
| Description | Anti-Dyskerin (Rabbit) |
| Alternate Names |
|
| Background Information | H/ACA ribonucleoprotein complex subunit 4 (UniProt: O60832; also known as CBF5 homolog, Dyskerin, Nopp140-associated protein of 57 kDa, Nucleolar protein NAP57, Nucleolar protein family A member 4, snoRNP protein DKC1) is encoded by the DKC1 (also known as NOLA4) gene (Gene ID: 1736) in human. Dyskerin is a highly conserved nucleolar protein that is involved with multiple cellular functions, such as protein expression, growth, proliferation, and nucleo-cytoplasmic shuttling. It helps in the stabilization of the telomerase RNA component, which allows for the proper functioning of telomerase enzymatic complex. It also modifies specific uridine residues of ribosomal RNA by converting them to pseudouridine, thus allowing proper ribosome processing and function. Point mutations in the DKC1 gene result in a rare inherited syndrome called X-linked dyskeratosis congenita, which is leads to early mortality due to progressive bone marrow failure and is characterized by the triad of reticulated skin hyperpigmentation, nail dystrophy, and mucosal leukoplakia. (Ref.: Montanaro, L (2010). J. Pathol. 222(4): 345-9). |
| Product Information | |
|---|---|
| Format | Affinity Purified |
| Presentation | Purified rabbit polyclonal antibody in buffer containing 20 mM Tris, 200 mM KCl, 1 mM EDTA, pH 8.5 with 0.02% sodium azide. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Anti-Dyskerin (Rabbit), Cat. No. ABE2587, is a highly specific rabbit polyclonal antibody that targets H/ACA ribonucleoprotein complex subunit 4 and has been tested for use in Immunofluorescence, Immunoprecipitation, and in Western Blotting. |
| Key Applications |
|
| Application Notes | Western Blotting Analysis: A 1:500 dilution from a representative lot detected Dyskerin in 10 µg of HeLa cell lysate. Immunoprecipitation Analysis: A representative lot recovered Dyskerin associated with active telomerase in telomerase-overexpressing HEK293T cells (Courtesy of the laboratories of Dr. Hilda Pickett and Dr. Tracy Bryan, Children s Medical Research Institute, Australia). Western Blotting Analysis: A representative lot detected Dyskerin in Western Blotting applications (OBrien, R., et. al. (2016). Cancer Res. 76(12):3604-17). Western Blotting Analysis: A representative lot detected Dyskerin in mid-S phase HEK293T cells. The signal depleted upon treatment with siRNA against Dyskerin. (Moye, A.L., et. al. (2015). Nat Commun. 6:7643). Immunofluorescence Analysis: A representative lot detected Dyskerin in HeLa cells. (telomerase positive) (Courtesy of the laboratories of Dr. Hilda Pickett and Dr. Tracy Bryan, Children s Medical Research Institute, Australia). Western Blotting Analysis: A representative lot detected Dyskerin in HEK293 Cell lysate. The signal was depleted upon treatment with siRNA against Dyskerin (Courtesy of the laboratories of Dr. Hilda Pickett and Dr. Tracy Bryan, Children s Medical Research Institute, Australia). |
| Biological Information | |
|---|---|
| Immunogen | KLH-conjugated linear peptide corresponding to 16 amino acids from the C-teminal half of human Dyskerin, isoform 1. |
| Epitope | unknown |
| Concentration | Please refer to lot specific datasheet. |
| Host | Rabbit |
| Specificity | This rabbit polyclonal antibody detects Dyskerin in human cells. It targets a 16 amino acid epitope within the C-terminal half of the Dyskerin protein. For immunoprecipitaion the use of HEPES or Tris bufer with KCl is highly recommended. |
| Isotype | IgG |
| Species Reactivity |
|
| Species Reactivity Note | Human. |
| Antibody Type | Polyclonal Antibody |
| Gene Symbol |
|
| Purification Method | Affinity Purified |
| UniProt Number |
|
| Molecular Weight | ~58 kDa observed; 57.67 kDa calculated. Uncharacterized bands may be observed in some lysate(s). |
| Product Usage Statements | |
|---|---|
| Quality Assurance | Evaluated by Western Blotting in SW480 cell lysate. Western Blotting Analysis: A 1:500 dilution of this antibody detected Dyskerin in 10 µg of SW480 cell lysate. |
| Usage Statement |
|
| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Stable for 1 year at 2-8°C from date of receipt. |
| Packaging Information | |
|---|---|
| Material Size | 200 µL |