产品名称
Anti-DKC1 antibody produced in rabbit, ~1.0 mg/mL, affinity isolated antibody
biological source
rabbit
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
form
buffered aqueous solution
mol wt
antigen ~57 kDa
species reactivity
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.
UniProt accession no.
shipped in
dry ice
storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
human ... DKC1(1736)
Application
Anti-DKC1 antibody has been used in immunoblotting.
Biochem/physiol Actions
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.
Disclaimer
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.
General description
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.
Immunogen
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.
Physical form
a solution in 0.01 M phosphate buffered saline pH 7.4, containing 15 mM sodium azide
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存储类别
10 - Combustible liquids
flash_point_f
Not applicable
flash_point_c
Not applicable
法规信息
新产品
此项目有
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)
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)
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)
Telomerase reverse-transcriptase homozygous mutations in autosomal recessive dyskeratosis congenita and Hoyeraal-Hreidarsson syndrome
Marrone A, et al.
Blood, 110(13), 4198-4205 (2007)
Erythrocyte scaffolding protein p55/MPP1 functions as an essential regulator of neutrophil polarity.
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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