Dyskerin Antibody (H-3): sc-373956

Dyskerin Antibody (H-3) is a mouse monoclonal IgG2a kappa light chain antibody that detects Dyskerin protein of mouse, rat, and human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). Anti-Dyskerin antibody (H-3) is available in both non-conjugated and various conjugated forms, including agarose, horseradish peroxidase (HRP), phycoerythrin (PE), fluorescein isothiocyanate (FITC), and multiple Alexa Fluor® conjugates. Dyskerin, also known as NAP57, plays a crucial role in the biogenesis of ribonucleoprotein complexes, specifically in the isomerization of uridine to pseudouridine, which is essential for proper functioning of ribosomal RNA and protein synthesis. This process maintains cellular homeostasis and ensures efficient translation, as pseudouridylation enhances the stability and functionality of RNA molecules. Dyskerin forms a small ribonucleoprotein particle with GAR1, NHP2, and Nop10, and these components localize to the dense fibrillar component of the nucleolus and nuclear Cajal bodies, highlighting their importance in nucleolar function and RNA processing. The dyskerin gene is located on chromosome Xq28, and mutations in this gene can disrupt normal nuclear localization, leading to Dyskeratosis congenita, a rare X-linked disorder characterized by symptoms such as cutaneous hyperpigmentation, nail dystrophy, testicular atrophy, and leukoplakia of the oral mucosa. The GAR1 gene is situated on chromosome 4q25, while the NHP2 gene, which encodes a 155-amino acid protein, is located on chromosome 5q35.3.

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Dyskerin Antibody (H-3) References:

1. Dyskerin localizes to the nucleolus and its mislocalization is unlikely to play a role in the pathogenesis of dyskeratosis congenita.  |  Heiss, NS., et al. 1999. Hum Mol Genet. 8: 2515-24. PMID: 10556300
2. In vitro assembly of human H/ACA small nucleolar RNPs reveals unique features of U17 and telomerase RNAs.  |  Dragon, F., et al. 2000. Mol Cell Biol. 20: 3037-48. PMID: 10757788
3. Human H/ACA small nucleolar RNPs and telomerase share evolutionarily conserved proteins NHP2 and NOP10.  |  Pogacić, V., et al. 2000. Mol Cell Biol. 20: 9028-40. PMID: 11074001
4. Immunopurified small nucleolar ribonucleoprotein particles pseudouridylate rRNA independently of their association with phosphorylated Nopp140.  |  Wang, C., et al. 2002. Mol Cell Biol. 22: 8457-66. PMID: 12446766
5. A dyskerin motif reactivates telomerase activity in X-linked dyskeratosis congenita and in telomerase-deficient human cells.  |  Machado-Pinilla, R., et al. 2008. Blood. 111: 2606-14. PMID: 18057229
6. Novel dyskerin-mediated mechanism of p53 inactivation through defective mRNA translation.  |  Montanaro, L., et al. 2010. Cancer Res. 70: 4767-77. PMID: 20501855
7. Dyskerin and cancer: more than telomerase. The defect in mRNA translation helps in explaining how a proliferative defect leads to cancer.  |  Montanaro, L. 2010. J Pathol. 222: 345-9. PMID: 20925138
8. A new human dyskerin isoform with cytoplasmic localization.  |  Angrisani, A., et al. 2011. Biochim Biophys Acta. 1810: 1361-8. PMID: 21820037
9. Multiscale in situ analysis of the role of dyskerin in lung cancer cells.  |  Fernandez-Garcia, I., et al. 2013. Integr Biol (Camb). 5: 402-13. PMID: 23233094
10. Severity of X-linked dyskeratosis congenita (DKCX) cellular defects is not directly related to dyskerin (DKC1) activity in ribosomal RNA biogenesis or mRNA translation.  |  Thumati, NR., et al. 2013. Hum Mutat. 34: 1698-707. PMID: 24115260
11. Discovery of new chromen-4-one derivatives as telomerase inhibitors through regulating expression of dyskerin.  |  Quan Wang, J., et al. 2018. J Enzyme Inhib Med Chem. 33: 1199-1211. PMID: 30132373
12. Mapping and characterization of the X-linked dyskeratosis congenita (DKC) gene.  |  Hassock, S., et al. 1999. Genomics. 55: 21-7. PMID: 9888995

Monoclonal antibodies for mammalian and non-mammalian protein targets represent essentially all targets covered by polyclonal antibodies. Over 8,300 of our antibodies are offered as ImmunoCruz^® conjugates which eliminates the need for a secondary antibody.

Santa Cruz Biotechnology antibodies have over 360,000 research citations. Anti-Dyskerin Antibody (H-3) has 27 citations in a variety of scientific publications.

Primary antibodies like Anti-Dyskerin Antibody (H-3) for mammalian target proteins are recommended for the detection of a range of mammalian species, primarily of mouse, rat and human species. Many mammalian antibodies are also ideal for veterinary research and reactive with ovine, porcine, caprine, feline, canine, bovine and equine protein targets. Santa Cruz Biotechnology primary monoclonal antibodies are suitable for laboratory research applications and are not intended for diagnostic or therapeutic use.

Dyskerin Antibody (H-3) Specifications

Alternate Product Name: Dyskerin

Product Clone Name: H-3

Classification: Primary Antibody

Clonality: Monoclonal Antibody

Antibody Isotype: IgG_2a kappa light chain

Amino Acids: 171-470

Epitope Target: Dyskerin

Epitope Species: human

Product Reactivity/Detection: Dyskerin

Species Reactivity/Detection: mouse, rat, human

Additional Mammalian Reactivity: N/A

Additional Non-Mammalian Reactivity: N/A

Applications: WB, IP, IF, ELISA

Vialing Information: Each vial contains 200 µg IgG_2a kappa light chain in 1.0 ml of PBS with < 0.1% sodium azide and 0.1% gelatin

Research Citations: 27 citations in various scientific publications

Available Conjugates/Forms for Dyskerin Antibody (H-3): AC: Agarose, AF488: Alexa Fluor^® 488, AF546: Alexa Fluor^® 546, AF594: Alexa Fluor^® 594, AF647: Alexa Fluor^® 647, AF680: Alexa Fluor^® 680, AF790: Alexa Fluor^® 790, FITC: Fluoroscein, HRP: Horseradish Peroxidase, PE: Phycoerythrin, X: TransCruz Reagent