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Key Documents

SAB4200611

Sigma-Aldrich

Anti-DKC1 antibody produced in rabbit

~1.0 mg/mL, affinity isolated antibody

Synonyme(s) :

Anti-CBF5, Anti-CBF5 homolog, Anti-DKC, Anti-H/ACA ribonucleoprotein complex subunit 4, Anti-NAP57, Anti-NOLA4, Anti-XAP101, Anti-cbf5p homolog, Anti-dyskeratosis congenita 1, Anti-dyskerin, Anti-nopp140-associated protein of 57 kDa, Anti-nucleolar protein family A member 4, Anti-snoRNP protein DKC1

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About This Item

Code UNSPSC :
12352203
Nomenclature NACRES :
NA.41

Source biologique

rabbit

Conjugué

unconjugated

Forme d'anticorps

affinity isolated antibody

Type de produit anticorps

primary antibodies

Clone

polyclonal

Forme

buffered aqueous solution

Poids mol.

antigen ~57 kDa

Espèces réactives

human, mouse

Concentration

~1.0 mg/mL

Technique(s)

immunoblotting: 0.5-1.0 μg/mL using whole extracts of mouse Hepa1-6 cells or nuclear extract of HeLa cells.

Numéro d'accès UniProt

Conditions d'expédition

dry ice

Température de stockage

−20°C

Modification post-traductionnelle de la cible

unmodified

Informations sur le gène

human ... DKC1(1736)

Description générale

Dyskerin is a nucleolar protein encoded by the DKC1 gene. This gene is a member of the H/ACA snoRNPs (small nucleolar ribonucleoproteins) gene family. The DKC1 gene lies in a tail-to-tail orientation with the palmitoylated erythrocyte membrane protein gene and is transcribed in a telomere to centromere direction.

Immunogène

peptide corresponding to the N-terminal region of human DKC1, conjugated to KLH. The corresponding sequence is identical in monkey, bovine, pig and dog and differs by a single amino acid in mouse and rat.

Application

Anti-DKC1 antibody has been used in immunoblotting.

Actions biochimiques/physiologiques

Dyskerin and the three NOLA proteins localize to the dense fibrillar components of nucleoli and to coiled (Cajal) bodies in the nucleus. Mutations in the DKC1 gene cause X-linked dyskeratosis congenita, aplastic anemia and progressive bone marrow failure in most cases. It also leads to a disease resulting in reticulate skin pigmentation, mucosal leukoplakia, nail dystrophy. It also causes Hoyeraal-Hreidarsson syndrome, which is a more severe form of dyskeratosis congenita. The overexpression of dyskerin has been observed in many cancers including neuroblastoma, colorectal cancer, lymphoma, breast cancer, melanoma, prostate cancer, ovarian carcinoma and hepatocellular carcinoma.

Forme physique

a solution in 0.01 M phosphate buffered saline pH 7.4, containing 15 mM sodium azide

Clause de non-responsabilité

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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Code de la classe de stockage

10 - Combustible liquids

Point d'éclair (°F)

Not applicable

Point d'éclair (°C)

Not applicable


Certificats d'analyse (COA)

Recherchez un Certificats d'analyse (COA) en saisissant le numéro de lot du produit. Les numéros de lot figurent sur l'étiquette du produit après les mots "Lot" ou "Batch".

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Consulter la Bibliothèque de documents

Dyskerin overexpression in human hepatocellular carcinoma is associated with advanced clinical stage and poor patient prognosis
Liu B, et al.
PLoS ONE, 7(8), e43147-e43147 (2012)
Telomerase reverse-transcriptase homozygous mutations in autosomal recessive dyskeratosis congenita and Hoyeraal-Hreidarsson syndrome
Marrone A, et al.
Blood, 110(13), 4198-4205 (2007)
An enhanced H/ACA RNP assembly mechanism for human telomerase RNA
Egan E D and Collins K
Molecular and Cellular Biology, 32(13), 2428-2439 (2012)
Mouse dyskerin mutations affect accumulation of telomerase RNA and small nucleolar RNA, telomerase activity, and ribosomal RNA processing
Mochizuki Y, et al.
Proceedings of the National Academy of Sciences of the USA, 101(29), 10756-10761 (2004)
Brendan J Quinn et al.
Proceedings of the National Academy of Sciences of the United States of America, 106(47), 19842-19847 (2009-11-10)
As mediators of innate immunity, neutrophils respond to chemoattractants by adopting a highly polarized morphology. Efficient chemotaxis requires the formation of one prominent pseudopod at the cell front characterized by actin polymerization, while local inhibition suppresses the formation of rear

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