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AB152335

Recombinant Human DKC1/Dyskerin protein (GST tag N-Terminus)

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Recombinant Human DKC1/Dyskerin protein (GST tag N-Terminus) is a Human Fragment protein, in the 321 to 420 aa range, expressed in Wheat germ, suitable for SDS-PAGE, ELISA, WB.

View Alternative Names

NOLA4, DKC1, H/ACA ribonucleoprotein complex subunit DKC1, CBF5 homolog, Dyskerin, Nopp140-associated protein of 57 kDa, Nucleolar protein NAP57, Nucleolar protein family A member 4, snoRNP protein DKC1

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SDS-PAGE - Recombinant Human DKC1/Dyskerin protein (GST tag N-Terminus) (AB152335)
  • SDS-PAGE

Unknown

SDS-PAGE - Recombinant Human DKC1/Dyskerin protein (GST tag N-Terminus) (AB152335)

12.5% SDS-PAGE analysis of ab152335 stained with Coomassie Blue.

Key facts

Expression system

Wheat germ

Tags

GST tag N-Terminus

Applications

SDS-PAGE, ELISA, WB

applications

Biologically active

No

Accession

O60832

Animal free

No

Carrier free

No

Species

Human

Storage buffer

pH: 8 Constituents: 0.79% Tris HCl, 0.31% Glutathione

storage-buffer

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Complementary Products

Primary Antibodies

AB156877

Anti-DKC1/Dyskerin antibody [EPR10399]

RabMAb|Recombinant

Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-DKC1/Dyskerin antibody [EPR10399] (AB156877)

  • 5
  • 1
Secondary Antibodies

AB150077

Goat Anti-Rabbit IgG H&L (Alexa Fluor® 488)

Immunocytochemistry/ Immunofluorescence - Goat Anti-Rabbit IgG H&L (Alexa Fluor® 488) (AB150077)

  • 5
  • 20
Reagents, Controls & Accessories

AB7481

Normal Goat Serum

Immunohistochemistry (Frozen sections) - Normal Goat Serum (AB7481)

  • 5
  • 3
Primary Antibodies

AB9485

Anti-GAPDH antibody - Loading Control

BOND RX™ Validated|Lab Essentials

Immunocytochemistry/ Immunofluorescence - Anti-GAPDH antibody - Loading Control (AB9485)

  • 5
  • 88
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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "SDS-PAGE": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" }, "ELISA": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" }, "WB": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }
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Product details

This product was previously labelled as DKC1.
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Sequence info

[{"sequence":"LRYEDGIEVNQEIVVITTKGEAICMAIALMTTAVISTCDHGIVAKIKRVIMERDTYPRKWGLGPKASQKKLMIKQGLLDKHGKPTDSTPATWKQEYVDYS","proteinLength":"Fragment","predictedMolecularWeight":"36.63 kDa","actualMolecularWeight":null,"aminoAcidEnd":420,"aminoAcidStart":321,"nature":"Recombinant","expressionSystem":"Wheat germ","accessionNumber":"O60832","tags":[{"tag":"GST","terminus":"N-Terminus"}]}]
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Properties and storage information

Shipped at conditions
Dry Ice
Appropriate short-term storage conditions
-80°C
Appropriate long-term storage conditions
-80°C
Aliquoting information
Upon delivery aliquot
Storage information
Avoid freeze / thaw cycle
False
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Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

DKC1 also known as Dyskerin is a protein weighing approximately 57 kDa. It shows high expression in the nucleolus of cells aiding in key cellular processes. Dyskerin acts mechanically as a pseudouridine synthase which catalyzes the isomerization of uridine to pseudouridine in RNA molecules. This activity plays a significant role in rRNA modification and stabilization. Also Dyskerin functions as a scaffold within ribonucleoprotein complexes which is fundamental for ribosome biogenesis.
Biological function summary

Dyskerin is essential for maintaining the integrity of ribosomal RNA through its action within the H/ACA ribonucleoprotein complex. The protein binds H/ACA box small nucleolar RNAs (snoRNAs) which guides it to specific uridine residues in rRNA thereby modifying them to pseudouridine. These modifications have an important role in forming functional ribosomes. Additionally Dyskerin contributes to the stabilization of telomerase RNA component which impacts telomere maintenance and length regulation.

Pathways

The role of Dyskerin in ribosome biogenesis directly connects it to cellular growth and protein synthesis pathways. It associates with NOP10 GAR1 and NHP2 in the H/ACA ribonucleoprotein complex contributing to the processing of precursor rRNA. Dyskerin also associates with telomerase complex proteins like TERT functioning in the telomere maintenance pathway. In these pathways its activity ensures the accurate synthesis of proteins and the preservation of genomic stability.

Dyskerin mutations relate to X-linked dyskeratosis congenita a condition characterized by impaired telomere maintenance leading to bone marrow failure. Additionally abnormal levels of Dyskerin have associations with certain cancers due to its role in rRNA processing and telomere regulation which can drive malignant transformation. Proteins like TERC which forms part of the telomerase complex are frequently involved in these Dyskerin-related disorders highlighting Dyskerin's critical involvement in cellular division and stability.
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Specifications

Form

Liquid

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General info

Function

Isoform 1. Catalytic subunit of H/ACA small nucleolar ribonucleoprotein (H/ACA snoRNP) complex, which catalyzes pseudouridylation of rRNA (PubMed : 25219674, PubMed : 32554502). This involves the isomerization of uridine such that the ribose is subsequently attached to C5, instead of the normal N1 (PubMed : 25219674). Each rRNA can contain up to 100 pseudouridine ('psi') residues, which may serve to stabilize the conformation of rRNAs. Required for ribosome biogenesis and telomere maintenance (PubMed : 19179534, PubMed : 25219674). Also required for correct processing or intranuclear trafficking of TERC, the RNA component of the telomerase reverse transcriptase (TERT) holoenzyme (PubMed : 19179534).. Isoform 3. Promotes cell to cell and cell to substratum adhesion, increases the cell proliferation rate and leads to cytokeratin hyper-expression.

Sequence similarities

Belongs to the pseudouridine synthase TruB family.

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Product protocols

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Target data

Isoform 1. Catalytic subunit of H/ACA small nucleolar ribonucleoprotein (H/ACA snoRNP) complex, which catalyzes pseudouridylation of rRNA (PubMed : 25219674, PubMed : 32554502). This involves the isomerization of uridine such that the ribose is subsequently attached to C5, instead of the normal N1 (PubMed : 25219674). Each rRNA can contain up to 100 pseudouridine ('psi') residues, which may serve to stabilize the conformation of rRNAs. Required for ribosome biogenesis and telomere maintenance (PubMed : 19179534, PubMed : 25219674). Also required for correct processing or intranuclear trafficking of TERC, the RNA component of the telomerase reverse transcriptase (TERT) holoenzyme (PubMed : 19179534).. Isoform 3. Promotes cell to cell and cell to substratum adhesion, increases the cell proliferation rate and leads to cytokeratin hyper-expression.
See full target information DKC1
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