AB152335

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

Name: Recombinant Human DKC1/Dyskerin protein (ab152335) | Abcam
Brand: Abcam Limited
SKU: AB152335
Price: 118000 JPY
Availability: InStock

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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.

別名を表示する

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

1 Images

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

発現系

Wheat germ

アプリケーション

SDS-PAGE, ELISA, WB

applications

生物活性

アクセッション番号

O60832

アニマルフリー

キャリアフリー

種

Human

バッファー組成

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

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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":"" }, "ELISA": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"" }, "WB": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"" } } }

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製品の詳細

This product was previously labelled as DKC1.

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配列情報

[{"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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出荷温度及び保存条件

出荷温度

Dry Ice

短期保存温度

-80°C

長期保存温度

-80°C

分注に関する情報

Upon delivery aliquot

保管に関する情報

Avoid freeze / thaw cycle

False

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補足情報

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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製品の性状

製品の状態

Liquid

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一般的な情報

機能

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.

配列の類似性

Belongs to the pseudouridine synthase TruB family.

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製品プロトコール

Visit the General protocols
Visit the Troubleshooting

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ターゲットの情報

See full target information DKC1

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Abcam product promise

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