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Rabbit Anti-DKC1 Polyclonal Antibody#abs136186

Please note that the price mentioned above is for reference purposes only. For detailed pricing information, we kindly request you to get in touch with our sales representative, Vecent. Western blot analysis of extracts of variouscell lines, using DKC1 antibody. This product is for research use...

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Description

Catalog-specification	Delivery time	USD price
abs136186-100ug	1-2 Weeks	301.0
abs136186-50ug	1-2 Weeks	201.0

Please note that the price mentioned above is for reference purposes only. For detailed pricing information, we kindly request you to get in touch with our sales representative, Vecent.

Overview
catalog	abs136186
Description	This gene is a member of the H/ACA snoRNPs gene family that plays a vital role in rRNA processing and modification. This family is further categorized into two subfamilies: C/D and H/ACA and comprises some important proteins such as NOLA1, 2, and 3. The product of this gene and the three NOLA proteins are localized to the nucleoli's dense fibrillar components and coiled bodies in the nucleus. Depletion of any of the four proteins results in impaired 18S rRNA production and rRNA pseudouridylation. Additionally, these proteins are crucial components of the telomerase complex. The encoded protein shares a strong resemblance with Cbf5p of Saccharomyces cerevisiae and Nop60B of Drosophila melanogaster. The gene is situated tail-to-tail with the palmitoylated erythrocyte membrane protein gene, transcribed in a telomere to centromere direction. This gene is found to have nucleotide substitutions and trinucleotide repeat polymorphisms. Mutations in this gene result in X-linked dyskeratosis congenita leading to progressive bone marrow failure, reticulate skin pigmentation, mucosal leukoplakia, and nail dystrophy. Additionally, Hoyeraal-Hreidarsson syndrome, which is a more severe form of dyskeratosis congenita, is also caused by mutations in this gene. This gene has two transcript variants that code for different isoforms. RefSeq, Dec 2008, provided all these details.
Other names	CBF5 is a homolog of CBF5, also known as Cbf5p. It is associated with Dyskeratosis congenita 1, a genetic disorder. The protein, also referred to as DKC1, is responsible for the H/ACA ribonucleoprotein complex subunit 4. It is also known as Dyskerin and is encoded by the DKC1 gene in humans. Additionally, it is associated with NAP57 and NOLA4, which are other names for the same protein. Nopp140-associated protein of 57 kDa or NAP57, as well as XAP101, are alternative names for this nucleolar protein. Overall, DKC1 plays a crucial role in various cellular processes.
Source	Rabbit
Specificity	Total DKC1 can be detected by the DKC1 Antibody at its endogenous levels. This antibody is specifically designed to target and bind to DKC1.
Species Reactivity	Human;Mouse;Rat
Antigen	DKC1
Application	WB 1:500-1:2000, IHC 1:50-1:200, IF/ICC 1:100-1:500, ELISA(peptide) 1:20000-1:40000
Immunogen	A synthesized peptide derived from human DKC1.
MW	58kDa
Properties
Concentration	1mg/ml
purification	The antiserum was purified by peptide affinity chromatography using SulfoLink™ Coupling Resin .
Clonality	Polyclonal Antibody
Stability & Storage	Store at -20 °C for one year. Avoid repeated freeze/thaw cycles
Storage buffer	Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.Store at -20 °C.Stable for 12 months from date of receipt.
Target
Background	Isoform 1: Required for ribosome biogenesis and telomere maintenance. Probable catalytic subunit of H/ACA small nucleolar ribonucleoprotein (H/ACA snoRNP) complex, which catalyzes pseudouridylation of rRNA. This involves the isomerization of uridine such that the ribose is subsequently attached to C5, instead of the normal N1. Each rRNA can contain up to 100 pseudouridine ('psi') residues, which may serve to stabilize the conformation of rRNAs. Also required for correct processing or intranuclear trafficking of TERC, the RNA component of the telomerase reverse transcriptase (TERT) holoenzyme.
Tissue specificity	Ubiquitously expressed.
Celluar localization	Nucleus;
UniPort	O60832