Western Blot (WB)
(Western Blot: Sample: Lane1: Human HepG2 Cells; Lane2: Human Jurkat Cells; Lane3: Human Hela Cells.)
Rabbit anti-Human Dyskerin Polyclonal Antibody | anti-DKC antibody
Dyskerin (DKC) Polyclonal Antibody
Immunohistochemistry in formalin fixed frozen section: 1:50-500
Enzyme-linked Immunosorbent Assay: 1:100-200
Usage: 10uL per well when 3,3'-Diaminobenzidine(DAB) as the substrate.
5uL per well when used in enhanced chemilumescent (ECL).
Note: The quality control is specifically manufactured as the positive control.Not used for other purposes.
Loading Buffer: 100mM Tris(pH8.8), 2% SDS, 200mM NaCl, 50% glycerol,BPB 0.01%, NaN3 0.02%.
Western Blot (WB)
(Western Blot: Sample: Lane1: Human HepG2 Cells; Lane2: Human Jurkat Cells; Lane3: Human Hela Cells.)
Western Blot (WB)
(Western Blot: Sample: Lane1: Human HepG2 Cells; Lane2: Human Jurkat Cells; Lane3: Human Hela Cells.)
Western Blot (WB)
(Western Blot: Sample: Recombinant DKC, Human.)
Western Blot (WB)
(Western Blot: Sample: Recombinant DKC, Human.)
Immunohistochemistry (IHC)
(DAB staining on fromalin fixed paraffin- embedded liver tissue))
Immunohistochemistry (IHC)
(DAB staining on fromalin fixed paraffin- embedded liver tissue))
Knockout Validation
(Knockout Validation: Lane 1: Wild-type HepG2 cell lysate; Lane 2: DKC knockout HepG2 cell lysate; Predicted MW: 58,48kd Observed MW: 58kd Primary Ab: 1ug/ml Rabbit Anti-Human DKC Antibody Second Ab: 0.2ug/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody (#MBS2086047))
Knockout Validation
(Knockout Validation: Lane 1: Wild-type HepG2 cell lysate; Lane 2: DKC knockout HepG2 cell lysate; Predicted MW: 58,48kd Observed MW: 58kd Primary Ab: 1ug/ml Rabbit Anti-Human DKC Antibody Second Ab: 0.2ug/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody (#MBS2086047))
NCBI and Uniprot Product Information
NCBI Description
This gene functions in two distinct complexes. It plays an active role in telomerase stabilization and maintenance, as well as recognition of snoRNAs containing H/ACA sequences which provides stability during biogenesis and assembly into H/ACA small nucleolar RNA ribonucleoproteins (snoRNPs). This gene is highly conserved and widely expressed, and may play additional roles in nucleo-cytoplasmic shuttling, DNA damage response, and cell adhesion. Mutations have been associated with X-linked dyskeratosis congenita. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2014]
Uniprot Description
dyskerin: 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. Defects in DKC1 are a cause of dyskeratosis congenita X- linked recessive (XDKC). XDKC is a rare, progressive bone marrow failure syndrome characterized by the triad of reticulated skin hyperpigmentation, nail dystrophy, and mucosal leukoplakia. Early mortality is often associated with bone marrow failure, infections, fatal pulmonary complications, or malignancy. Defects in DKC1 are the cause of Hoyeraal-Hreidarsson syndrome (HHS). HHS is a multisystem disorder affecting males and is characterized by aplastic anemia, immunodeficiency, microcephaly, cerebellar hypoplasia, and growth retardation. Belongs to the pseudouridine synthase TruB family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: EC 5.4.99.-; Isomerase; Lyase; Nucleolus; RNA processing; RNA-binding
Chromosomal Location of Human Ortholog: Xq28
Cellular Component: box H/ACA scaRNP complex; cytoplasm; fibrillar center; nucleolus; nucleoplasm; nucleus; telomerase holoenzyme complex
Molecular Function: box H/ACA snoRNA binding; protein binding; pseudouridine synthase activity; RNA binding; telomerase activity; telomerase RNA binding
Biological Process: box H/ACA snoRNA 3'-end processing; cell proliferation; mRNA pseudouridine synthesis; positive regulation of establishment of protein localization to telomere; positive regulation of telomerase activity; positive regulation of telomerase RNA localization to Cajal body; positive regulation of telomere maintenance via telomerase; regulation of telomerase RNA localization to Cajal body; RNA processing; rRNA processing; rRNA pseudouridine synthesis; snRNA pseudouridine synthesis; telomere maintenance via telomerase
Disease: Dyskeratosis Congenita, X-linked
Research Articles on DKC
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Product Notes
The DKC dkc1 (Catalog #AAA2032153) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Dyskerin (DKC) Polyclonal Antibody reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's Dyskerin can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (EIA). Western Blot: 1:50-400 Immunohistochemistry in formalin fixed frozen section: 1:50-500 Enzyme-linked Immunosorbent Assay: 1:100-200. Researchers should empirically determine the suitability of the DKC dkc1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Dyskerin, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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