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) (AP)

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.\n\nApplications:\nSuitable for use in Western Blot, Immunohistochemistry, Immunofluorescence, ELISA\n\nRecommended Dilution:\nELISA: 1:1,000\nWestern Blot: 1:100-500\nImmunohistochemistry: 1:50-100\nImmunofluorescence: 1:10-50\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Do not freeze alkaline phosphatase conjugates which could result in a substantial loss of enzymatic activity. Light sensitive. Aliquots are stable for 6 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

034655-AP

Size

200ul

Applications

ELISA, IF, IHC, WB

Hosts

Rabbit

Reactivities

Hum, Mouse

Form

Supplied as a liquid in PBS, pH 7.2. Contains ~0.09% sodium azide. Conjugated to Alkaline Phosphatase.

P Type

Pab

Purity

Purified by Protein A affinity chromatography.

Isotype

IgG

References

Rostamiani, K., et al. Blood Cells Mol. Dis. 44 (2), 88 (2010) :\nGrozdanov, P.N., et al. Hum. Mol. Genet. 18(23):4546-4551(2009)\nAshbridge, B., et al. Biochemistry 48(46):10858-10865(2009)\nSieron, P., et al. Br. J. Cancer 101(8):1410-1416(2009)

Additional Info

Human, mouse

Alternative Names

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