SAB4200611

Anti-DKC1 antibody produced in rabbit

~1.0 mg/mL, affinity isolated antibody

• Synonym(s): Anti-CBF5, Anti-CBF5 homolog, Anti-DKC, Anti-H/ACA ribonucleoprotein complex subunit 4, Anti-NAP57, Anti-NOLA4, Anti-XAP101, Anti-cbf5p homolog, Anti-dyskeratosis congenita 1, Anti-dyskerin, Anti-nopp140-associated protein of 57 kDa, Anti-nucleolar protein family A member 4, Anti-snoRNP protein DKC1
• UNSPSC Code: 12352203
• NACRES: NA.41

Properties

• biological source: rabbit
• conjugate: unconjugated
• antibody form: affinity isolated antibody
• antibody product type: primary antibodies
• clone: polyclonal
• form: buffered aqueous solution
• mol wt: antigen ~57 kDa
• species reactivity: human, mouse
• concentration: ~1.0 mg/mL
• technique(s): immunoblotting: 0.5-1.0 μg/mL using whole extracts of mouse Hepa1-6 cells or nuclear extract of HeLa cells.
• UniProt accession no.: O60832
• shipped in: dry ice
• storage temp.: −20°C
• target post-translational modification: unmodified
• Gene Information: human ... DKC1(1736)

Description

General description

Dyskerin is a nucleolar protein encoded by the DKC1 gene. This gene is a member of the H/ACA snoRNPs (small nucleolar ribonucleoproteins) gene family. The DKC1 gene lies in a tail-to-tail orientation with the palmitoylated erythrocyte membrane protein gene and is transcribed in a telomere to centromere direction.

Immunogen

peptide corresponding to the N-terminal region of human DKC1, conjugated to KLH. The corresponding sequence is identical in monkey, bovine, pig and dog and differs by a single amino acid in mouse and rat.

Application

Anti-DKC1 antibody has been used in immunoblotting.

Biochem/physiol Actions

Dyskerin and the three NOLA proteins localize to the dense fibrillar components of nucleoli and to coiled (Cajal) bodies in the nucleus. Mutations in the DKC1 gene cause X-linked dyskeratosis congenita, aplastic anemia and progressive bone marrow failure in most cases. It also leads to a disease resulting in reticulate skin pigmentation, mucosal leukoplakia, nail dystrophy. It also causes Hoyeraal-Hreidarsson syndrome, which is a more severe form of dyskeratosis congenita. The overexpression of dyskerin has been observed in many cancers including neuroblastoma, colorectal cancer, lymphoma, breast cancer, melanoma, prostate cancer, ovarian carcinoma and hepatocellular carcinoma.

Physical form

a solution in 0.01 M phosphate buffered saline pH 7.4, containing 15 mM sodium azide

Disclaimer

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Safety Information

• Storage Class Code: 10 - Combustible liquids
• Flash Point(F): Not applicable
• Flash Point(C): Not applicable