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anti-DKC1 antibody :: Goat DKC1 Polyclonal Antibody

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Catalog # MBS420483
Unit / Price
  0.1 mg  /  $300 +1 FREE 8GB USB
Immunohistochemistry (IHC)
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
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.
Immunogen Sequence Length
Immunogen Sequence
Species Reactivity
Tested: Human; Expected from sequence similarity: Human, Mouse
Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.
100ug specific antibody in 200ul (lot specific)
Peptide with sequence C-KRKRESESESDETPP, from the internal region of the protein sequence according to NP_001354.1.
Internal region
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.
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.

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
NCBI/Uniprot data below describe general gene information for DKC1. It may not necessarily be applicable to this product.
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
47,603 Da
NCBI Official Full Name
H/ACA ribonucleoprotein complex subunit 4 isoform 1
NCBI Official Synonym Full Names
dyskerin pseudouridine synthase 1
NCBI Official Symbol
NCBI Official Synonym Symbols
  [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
UniProt Synonym Gene Names
NOLA4  [Similar Products]
UniProt Entry Name
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]
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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