522279
DKC1
Größe
£665.00
- SKU:
- 522279
- Reinheit:
- Purified by immunoaffinity chromatography.
- Lagerbedingungen:
- -20°C
- Hersteller:
- United States Biological
- Host:
- Rabbit
- Immunogen:
- Synthetic peptide corresponding to human DKC1.
- Format:
- Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 50% glycerol.
- Weitere Details:
- 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. Applications: Suitable for use in Western Blot, Immunohistochemistry, Immunofluorescence/Immunocytochemistry and ELISA. Other applications not tested. Recommended Dilution: Western Blot: 1:500-1:2000, incubate membrane with diluted primary antibody in TBS, 5% milk, 0.1% Tween 20 at 4°C with gentle shaking, overnight. Immunohistochemistry: 1:50-1:200 Immunofluorescence (IF-IC): 1:100-1:500 Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months after receipt. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
- Versandbedingungen:
- Blue Ice