abx001537

antibody from Abbexa

Targeting: DKC1 Cbf5, DKC, dyskerin, NAP57, NOLA4, XAP101

Western blot (Recommended by provider)

Immunocytochemistry (Recommended by provider)

Immunohistochemistry (Recommended by provider)

Antibody Data

Product number

abx001537

Provider

Abbexa

Product name

H/ACA Ribonucleoprotein Complex Subunit DKC1 (DKC1) Antibody

Antibody type

Polyclonal

Description

DKC1 Antibody is a Rabbit Polyclonal antibody against DKC1. This gene is a member of the H/ACA snoRNPs (small nucleolar ribonucleoproteins) gene family. snoRNPs are involved in various aspects of rRNA processing and modification and have been classified into two families: C/D and H/ACA. The H/ACA snoRNPs also include the NOLA1, 2 and 3 proteins. The protein encoded by this gene and the three NOLA proteins localize to the dense fibrillar components of nucleoli and to coiled (Cajal) bodies in the nucleus. Both 18S rRNA production and rRNA pseudouridylation are impaired if any one of the four proteins is depleted. These four H/ACA snoRNP proteins are also components of the telomerase complex. The protein encoded by this gene is related to the Saccharomyces cerevisiae Cbf5p and Drosophila melanogaster Nop60B proteins. The gene lies in a tail-to-tail orientation with the palmitoylated erythrocyte membrane protein gene and is transcribed in a telomere to centromere direction. Both nucleotide substitutions and single trinucleotide repeat polymorphisms have been found in this gene. Mutations in this gene cause X-linked dyskeratosis congenita, a disease resulting in reticulate skin pigmentation, mucosal leukoplakia, nail dystrophy, and progressive bone marrow failure in most cases. Mutations in this gene also cause Hoyeraal-Hreidarsson syndrome, which is a more severe form of dyskeratosis congenita. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Dec 2008].

Reactivity

Human, Mouse, Rat

Host

Rabbit

Conjugate

Unconjugated

Isotype

IgG

Vial size

100 µl

Concentration

> 1 mg/ml

Storage

Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles.