Rabbit Anti-DKC (Dyskerin, DKC1, NAP57, NOLA4, NAP57, Dyskeratosis Congenita 1, H/ACA Ribonucleoprotein Complex Subunit 4, Nopp140-associated protein of 57kD, Nucleolar protein family A member 4)
Dyskeratosis congenita 1, dyskerin, is a member of the H/ACA snoRNPs (small nucleolar ribonucleoproteins). 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. 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 (MPP1) gene and is transcribed in a telomere to centromere direction. Both nucleotide substitutions and single trinucleotide repeat polymorphisms have been found in this gene.
Applications
Suitable for use in ELISA, Western Blot, Immunohistochemistry, Immunocytochemistry. Other applications not tested.
Recommended Dilution
ELISA: 1:100-1:5000 Western Blot: 1:50-400 Immunohistochemistry (frozen): 1:50-500 Immunohistochemistry (paraffin): 1:10-100 Immunocytochemistry: 1:50-500 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.
Immunogen
Recombinant protein corresponding to Arg298-Leu465 of human DKC, expressed in E. coli.
Form
Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 50% glycerol.
Purity
Purified by immunoaffinity chromatography.
Specificity
Recognizes human DKC.
