Dyskerin, Polyclonal Antibody

DKC1; NAP57; NOLA4; NAP57; Dyskeratosis Congenita 1; H/ACA Ribonucleoprotein Complex Subunit 4; Nopp140-associated protein of 57 kDa; Nucleolar protein family A member 4

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

The antibody is a rabbit polyclonal antibody raised against DKC. It has been selected for its ability to recognize DKC in immunohistochemical staining and western blotting.

Affinity Chromatography

Supplied as solution form in PBS, pH7.4 containing 0.02% NaN3, 50% glycerol.

200ug/ml (lot specific)

Store at 4 degree C for frequent use. Stored at -20 degree C to -80 degree C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customer’s specifications, please inquire.

Small volumes of anti-DKC antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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

Western Blot: Sample: Recombinant protein.

DAB staining on fromalin fixed paraffin- embedded liver tissue)

56,615 Da

dyskerin pseudouridine synthase 1

H/ACA ribonucleoprotein complex subunit 4

H/ACA ribonucleoprotein complex subunit 4

Nap57  [Similar Products]

nucleolar protein expressed in liver; colocalized with Nopp140 protein; may be involved with rRNA processing [RGD, Feb 2006]

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: Xq37

Cellular Component: Cajal body; fibrillar center; nucleolus; nucleoplasm; nucleus; telomerase holoenzyme complex

Molecular Function: protein binding; pseudouridine synthase activity; RNA binding; telomerase activity

Biological Process: box H/ACA snoRNA 3'-end processing; box H/ACA snoRNA metabolic process; positive regulation of cell proliferation; positive regulation of telomerase activity; positive regulation of telomere maintenance via telomerase; rRNA pseudouridine synthesis; snoRNA guided rRNA pseudouridine synthesis; snRNA pseudouridine synthesis; telomere maintenance via telomerase

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