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anti-DKC1 antibody :: Goat DKC1 Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS420483
Immunohistochemistry (IHC)
Unit / Price
0.1 mg  /  $300 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

DKC1, Polyclonal Antibody

 Also Known As   

Goat anti-DKC1 Antibody

 Product Synonym Names    DKC1; dyskeratosis congenita 1, dyskerin; DKC; NAP57; NOLA4; XAP101; DKC1 antibody; dyskeratosis congenita 1; dyskerin antibody; DKC antibody; NAP57 antibody; NOLA4 antibody; XAP101 antibody
 Product Gene Name   

anti-DKC1 antibody

[Similar Products]
 Antibody/Peptide Pairs    DKC1 peptide (MBS425350) is used for blocking the activity of DKC1 antibody (MBS420483)
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 Immunogen Sequence    KRKRESESES DETPP
 OMIM    300126
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 Clonality    Polyclonal
 Host    Goat
 Species Reactivity    Tested: Human; Expected from sequence similarity: Human, Mouse
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 Purity/Purification    Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
 Form/Format    Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.
 Concentration    100ug specific antibody in 200ul (lot specific)
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 Immunogen    Peptide with sequence C-KRKRESESESDETPP, from the internal region of the protein sequence according to NP_001354.1.
 Epitope    Internal region
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 Preparation and Storage    Aliquot and store at -20 degree C. Minimize freezing and thawing.
 ISO Certification    Manufactured in an ISO 9001:2015 Certified Laboratory.
 Other Notes    Small volumes of anti-DKC1 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.
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 Applications Tested/Suitable for anti-DKC1 antibody   

Peptide ELISA (EIA), Immunohistochemistry (IHC)

 Application Notes for anti-DKC1 antibody    Peptide ELISA: Antibody detection limit dilution 1: 128000.
Immunohistochemistry: In paraffin embedded Human Spleen shows strong nuclear staining of select splenocytes. Recommended concentration, 5-10ug/ml.
Western Blot: Preliminary experiments gave an approx 70-75kDa band in Human Bone Marrow, Duodenum and Skin lysates after 0. 01ug/ml antibody staining. Please note that currently we cannot find an explanation in the literature for the band we observe given the calculated size of 57.7kDa according to NP_001354.1. The 70-75kDa band was successfully blocked by incubation with the immunizing peptide.
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 Immunohistochemistry (IHC) of anti-DKC1 antibody    (5ug/ml) staining of paraffin embedded Human Spleen. Steamed antigen retrieval with citrate buffer pH 6, AP-staining.
anti-DKC1 antibody Immunohistochemistry (IHC) (IHC) image
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NCBI/Uniprot data below describe general gene information for DKC1. It may not necessarily be applicable to this product.
 NCBI GI #    4503337
 NCBI GeneID    1736
 NCBI Accession #    NP_001354.1 [Other Products]
 NCBI GenBank Nucleotide #    NM_001363.4 [Other Products]
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 UniProt Secondary Accession #    O43845; Q96G67; Q9Y505; F5BSB3 [Other Products]
 UniProt Related Accession #    O60832 [Other Products]
 Molecular Weight    47,603 Da
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 NCBI Official Full Name    H/ACA ribonucleoprotein complex subunit 4 isoform 1
 NCBI Official Synonym Full Names    dyskerin pseudouridine synthase 1
 NCBI Official Symbol    DKC1 [Similar Products]
 NCBI Official Synonym Symbols   
DKC; CBF5; DKCX; NAP57; NOLA4; XAP101
[Similar Products]
 NCBI Protein Information    H/ACA ribonucleoprotein complex subunit 4
 UniProt Protein Name    H/ACA ribonucleoprotein complex subunit 4
 UniProt Synonym Protein Names   
CBF5 homolog; Dyskerin; Nopp140-associated protein of 57 kDa; Nucleolar protein NAP57; Nucleolar protein family A member 4; snoRNP protein DKC1
 UniProt Gene Name    DKC1 [Similar Products]
 UniProt Synonym Gene Names    NOLA4 [Similar Products]
 UniProt Entry Name    DKC1_HUMAN
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 NCBI Summary for DKC1    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]
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 UniProt Comments for DKC1    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: Nucleolus; RNA-binding; RNA processing; EC 5.4.99.-; Lyase; Isomerase

Chromosomal Location of Human Ortholog: Xq28

Cellular Component: cytoplasm; 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; cell proliferation; positive regulation of telomerase activity; positive regulation of telomere maintenance via telomerase; RNA processing; rRNA processing; rRNA pseudouridine synthesis; snRNA pseudouridine synthesis; telomere maintenance via telomerase

Disease: Dyskeratosis Congenita, X-linked
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 Research Articles on DKC1    1. Performed a comprehensive study of human dyskerin through structural, phylogenetic and bioinformatic analysis.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with anti-DKC1 antibodyPathways associated with anti-DKC1 antibody
 Reference Product  PubMed Publications
 NOP10 antibody  >35 publications with DKC1 and NOP10
 NHP2 antibody  >29 publications with DKC1 and NHP2
 GAR1 antibody  >10 publications with DKC1 and GAR1
 MKI67IP antibody  >1 publications with DKC1 and MKI67IP
 NOP58 antibody  >1 publications with DKC1 and NOP58
 FBL antibody  >1 publications with DKC1 and FBL
 Products by Pathway  Pathway Diagram
 Cell Cycle Pathway antibodies  Cell Cycle Pathway Diagram
 Chromosome Maintenance Pathway antibodies  Chromosome Maintenance Pathway Diagram
 Extension Of Telomeres Pathway antibodies  Extension Of Telomeres Pathway Diagram
 Gene Expression Pathway antibodies  Gene Expression Pathway Diagram
 H/ACA Ribonucleoprotein Complex Pathway antibodies  H/ACA Ribonucleoprotein Complex Pathway Diagram
 H/ACA Ribonucleoprotein Complex Pathway antibodies  H/ACA Ribonucleoprotein Complex Pathway Diagram
 Regulation Of Telomerase Pathway antibodies  Regulation Of Telomerase Pathway Diagram
 Ribosome Biogenesis In Eukaryotes Pathway antibodies  Ribosome Biogenesis In Eukaryotes Pathway Diagram
 Ribosome Biogenesis In Eukaryotes Pathway antibodies  Ribosome Biogenesis In Eukaryotes Pathway Diagram
 Telomere Extension By Telomerase Pathway antibodies  Telomere Extension By Telomerase Pathway Diagram
Diseases associated with anti-DKC1 antibodyOrgans/Tissues associated with anti-DKC1 antibody
 Disease Name  Pubmed Publications
 Dyskeratosis Congenita Antibodies  >170 publications with DKC1 and Dyskeratosis Congenita
 Anemia Antibodies  >39 publications with DKC1 and Anemia
 Fibrosis Antibodies  >15 publications with DKC1 and Fibrosis
 Nervous System Diseases Antibodies  >13 publications with DKC1 and Nervous System Diseases
 Carcinoma Antibodies  >10 publications with DKC1 and Carcinoma
 Disease Models, Animal Antibodies  >7 publications with DKC1 and Disease Models, Animal
 Breast Neoplasms Antibodies  >6 publications with DKC1 and Breast Neoplasms
 Lung Neoplasms Antibodies  >5 publications with DKC1 and Lung Neoplasms
 Cell Transformation, Neoplastic Antibodies  >4 publications with DKC1 and Cell Transformation, Neoplastic
 Precancerous Conditions Antibodies  >4 publications with DKC1 and Precancerous Conditions
 Organ/Tissue Name  Pubmed Publications
 Bone Antibodies  >99 publications with DKC1 and Bone
 Bone Marrow Antibodies  >97 publications with DKC1 and Bone Marrow
 Blood Antibodies  >47 publications with DKC1 and Blood
 Skin Antibodies  >36 publications with DKC1 and Skin
 Lung Antibodies  >16 publications with DKC1 and Lung
 Brain Antibodies  >11 publications with DKC1 and Brain
 Liver Antibodies  >11 publications with DKC1 and Liver
 Embryonic Tissue Antibodies  >6 publications with DKC1 and Embryonic Tissue
 Prostate Antibodies  >5 publications with DKC1 and Prostate
 Eye Antibodies  >3 publications with DKC1 and Eye
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