DKC1 antibody
Catalogue Number: GTX109000-GTX
| Manufacturer: | GeneTex |
| Preservative: | 0.01% Thimerosal |
| Physical state: | Liquid |
| Type: | Polyclonal Primary Antibody - Unconjugated |
| Alias: | dyskerin pseudouridine synthase 1 , CBF5 , DKC , DKCX , NAP57 , NOLA4 , XAP101 |
| Shipping Condition: | Blue Ice |
| Unit(s): | 100 ul, 25 ul |
| Host name: | Rabbit |
| Clone: | |
| Isotype: | IgG |
| Immunogen: | Recombinant protein encompassing a sequence within the center region of human DKC1. The exact sequence is proprietary. |
| Application: | ICC, IF, IHC-P, IP, WB |
Description
Description: 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. [provided by RefSeq]
Additional Text
Concentration
1 mg/ml
Gene ID
1736
Gene Name
DKC1
Uniprot ID
O60832
Purification
Affinity Purified
Molecular Weight
58
Antibody Clonality
Polyclonal
Note
For In vitro laboratory use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption
Application Notes
WB: 1:500-1:3000. ICC/IF: 1:100-1:1000. IHC-P: 1:100-1:1000. *Optimal dilutions/concentrations should be determined by the researcher.Not tested in other applications.
GTX109000 Datasheet
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