Dyskeratosis Congenita 1, Dyskerin (DKC1) (Internal Region) antibody

Details for Product No. ABIN374810
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Antigen
Synonyms
fv62a07, wu:fa28f10, wu:fc87a02, wu:fi24a05, wu:fv62a07, zgc:110395, DKC1, cbf5, dkc, nap57, nola4, xap101, CBF5, DKC, DKCX, NAP57, NOLA4, XAP101, BC068171, Nap57, dyskerin, AtCBF5, AtNAP57, homologue ... show more
fv62a07, wu:fa28f10, wu:fc87a02, wu:fi24a05, wu:fv62a07, zgc:110395, DKC1, cbf5, dkc, nap57, nola4, xap101, CBF5, DKC, DKCX, NAP57, NOLA4, XAP101, BC068171, Nap57, dyskerin, AtCBF5, AtNAP57, homologue of NAP57 show less
Epitope
Internal Region
(8), (7), (3), (2), (1), (1), (1), (1), (1), (1), (1)
Reactivity
Human, Mouse (Murine)
(58), (37), (31), (14), (13), (13), (13), (1), (1), (1), (1)
Host
Goat
(50), (7), (1)
Clonality
Polyclonal
Conjugate
Un-conjugated
(2), (2), (2), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1)
Application
Immunohistochemistry (Frozen Paraffin-embedded Sections) (IHC (frpe)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))
(43), (24), (22), (19), (14), (10), (10), (2), (1), (1), (1)
Pubmed 1 reference available
Quantity 0.1 mg
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Catalog No. ABIN374810
357.50 $
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Immunogen Peptide from the internal region of the protein sequence according to NP_001354.1 Genename: DKC1
Sequence C-KRKRESESES DETPP
Specificity This antibody detects Dyskerin.
Cross-Reactivity (Details) Species reactivity (expected):MouseSpecies reactivity (tested):Human
Purification Ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide
Alternative Name DKC1 / NOLA4
Background DKC1 (Dyskeratosis congenita 1) is a nucleolar protein, altered in dyskeratosis congenita, which carries out two separate functions, both fundamental for proliferating cells. One function is the pseudouridylation of ribosomal RNA (rRNA) molecules, necessary for their processing, and the other is the stabilization of the telomerase RNA component, necessary for telomerase activity. It is thought to be the catalytic subunit of the H/ACA small nucleolar ribonucleoprotein (H/ACA snoRNP) complex, which catalyzes pseudouridylation of rRNA. Each rRNA can contain up to 100 pseudouridine ('psi') residues, which may serve to stabilize the conformation of rRNAs. DKC1 is also required for correct processing or intranuclear trafficking of TERC, the RNA component of the telomerase reverse transcriptase (TERT) holoenzyme. Both 18S rRNA production and rRNA pseudouridylation are impaired if DKC1 is depleted.Synonyms: CBF5 homolog, Dyskerin, H/ACA ribonucleoprotein complex subunit 4, Nopp140-associated protein of 57 kDa, Nucleolar protein NAP57, Nucleolar protein family A member 4, snoRNP protein DKC1
Gene ID 1736
NCBI Accession NP_001354
UniProt O60832
Application Notes Peptide ELISA: antibody detection limit dilution 1: 128000. Western blot: Preliminary experiments gave an approx 70-75 kDa band in Human BoneMarrow, Duodenum and Skin lysates after 0.01 μg/mL antibody staining. Please note thatcurrently we cannot find an explanation in the literature for the band we observe given thecalculated size of 57.5 kDa according to NP_001354.1. The 70-75 kDa band was successfullyblocked by incubation with the immunizing peptide. We would appreciate any feedbackfrom people in the field - have any results been reported with other antibodies/lysates?Have any further splice variants/modified forms been reported?Immunohistochemistry: In paraffin embedded Human Spleen shows strong nuclear
Restrictions For Research Use only
Concentration 0.5 mg/mL
Buffer Tris saline, 0.02 % sodium azide, pH 7.3 with 0.5 % bovine serum albumin
Preservative Sodium azide
Precaution of Use This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Handling Advice Avoid repeated freezing and thawing.
Storage 4 °C/-20 °C
Storage Comment Store the antibody at 2 - 8 °C up to one month or (in aliquots) at -20 °C for longer.
Supplier Images
anti-Dyskeratosis Congenita 1, Dyskerin (DKC1) (Internal Region) antibody anti-Dyskeratosis Congenita 1, Dyskerin (DKC1) (Internal Region) antibody
Background publications Yoon, Peng, Brandenburger et al.: "Impaired control of IRES-mediated translation in X-linked dyskeratosis congenita." in: Science (New York, N.Y.), Vol. 312, Issue 5775, pp. 902-6, 2006 (PubMed).

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