DKC1 (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, NOLA4)

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 (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. Mutations in this gene cause X-linked dyskeratosis congenita disease.\n\nApplications:\nSuitable for use in ELISA and Immunohistochemistry. Other applications not tested.\n\nRecommended Dilution:\nELISA: 1:128,000\nImmunohistochemistry (Paraffin): 5-10ug/ml\nOptimal dilutions to be determined by the researcher.\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

D3790-15C

Size

100ug

Applications

ELISA, IHC

Hosts

Goat

Reactivities

Hum, Mouse

Form

Supplied as a liquid in Tris saline, 0.02% sodium azide, pH 7.3, 0.5% BSA.

P Type

Pab

Purity

Purified by immunoaffinity chromatography.

References

1. Yoon A, Peng G, Brandenburger Y, Zollo O, Xu W, Rego E, Ruggero D. Impaired control of IRES-mediated translation in X-linked dyskeratosis congenita. Science. 2006 May 12;312(5775):902-6. Erratum in: Science. 2006.

Additional Info

Recognizes human DKC1. Species sequence homology: Mouse.