DKC1 Rabbit Polyclonal Antibody

CAT#: TA327204

Rabbit anti-DKC1 Polyclonal Antibody


USD 365.00

3 Weeks*

Size
    • 100 ul

Product Images

Frequently bought together (2)
beta Actin Mouse Monoclonal Antibody, Clone OTI1, Loading Control
    • 30 ul

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Transient overexpression lysate of dyskeratosis congenita 1, dyskerin (DKC1), transcript variant 2
    • 100 ug

USD 436.00

Other products for "DKC1"

Specifications

Product Data
Applications ICC/IF, IHC, WB
Recommended Dilution WB 1:500 - 1:2000;IF 1:50- 1:200
Reactivities Human, Mouse, Rat
Host Rabbit
Isotype IgG
Clonality Polyclonal
Immunogen Recombinant protein of human DKC1
Formulation Store at -20C or -80C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3
Concentration lot specific
Purification Affinity purification
Conjugation Unconjugated
Storage Store at -20°C as received.
Stability Stable for 12 months from date of receipt.
Gene Name dyskerin pseudouridine synthase 1
Background 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.
Synonyms CBF5; DKC; DKCX; NAP57; NOLA4; XAP101
Reference Data
Protein Families Druggable Genome

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*Delivery time may vary from web posted schedule. Occasional delays may occur due to unforeseen complexities in the preparation of your product. International customers may expect an additional 1-2 weeks in shipping.