84-2009-08　［取扱停止］DKC1, ID (DKC1, NOLA4, H/ACA ribonucleoprotein complex subunit 4, CBF5 homolog, Dyskerin, Nopp140-associated protein of 57kD, Nucleolar protein NAP57, Nucleolar protein family A member 4, snoRNP protein DKC1) (PE) 200ul　034655-PE

特徴

• DKC1 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.
• Applications:Suitable for use in Western Blot, Immunohistochemistry, Immunofluorescence, FLISA
• Recommended Dilution:FLISA: 1:1,000Western Blot: 1:100-500Immunohistochemistry: 1:50-100Immunofluorescence: 1:10-50
• Storage and Stability:Store product at 4°C in the dark.
• DO NOT FREEZE! Stable at 4°C for 12 months after receipt as an undiluted liquid.
• Dilute required amount only prior to immediate use.
• Further dilutions can be made in assay buffer.
• Caution: PE conjugates are sensitive to light.
• For maximum recovery of product, centrifuge the original vial prior to removing the cap.
• Note: Applications are based on unconjugated antibody.