8OUE

The H/ACA RNP lobe of human telomerase with the dyskerin thumb loop in a semi-closed conformation

これはPDB形式変換不可エントリーです。

8OUE の概要

• エントリーDOI: 10.2210/pdb8oue/pdb
• 関連するPDBエントリー: 8OUF
• EMDBエントリー: 17190
• 分子名称: H/ACA ribonucleoprotein complex subunit DKC1, H/ACA ribonucleoprotein complex subunit 1, H/ACA ribonucleoprotein complex subunit 2, ... (6 entities in total)
• 機能のキーワード: telomerase, h/aca, pseudouridylation, ribonucleoprotein, rna binding protein
• 由来する生物種: Homo sapiens (human); 詳細
• タンパク質・核酸の鎖数: 10
• 化学式量合計: 415061.33

構造登録者

Ghanim, G.E.,Sekne, Z.,van Roon, A.M.M.,Balch, S.,Nguyen, T.H.D. (登録日: 2023-04-22, 公開日: 2024-01-31, 最終更新日: 2024-02-07)

主引用文献

Ghanim, G.E.,Sekne, Z.,Balch, S.,van Roon, A.M.,Nguyen, T.H.D.
2.7 angstrom cryo-EM structure of human telomerase H/ACA ribonucleoprotein.
Nat Commun, 15:746-746, 2024

PubMed Abstract: Telomerase is a ribonucleoprotein (RNP) enzyme that extends telomeric repeats at eukaryotic chromosome ends to counterbalance telomere loss caused by incomplete genome replication. Human telomerase is comprised of two distinct functional lobes tethered by telomerase RNA (hTR): a catalytic core, responsible for DNA extension; and a Hinge and ACA (H/ACA) box RNP, responsible for telomerase biogenesis. H/ACA RNPs also have a general role in pseudouridylation of spliceosomal and ribosomal RNAs, which is critical for the biogenesis of the spliceosome and ribosome. Much of our structural understanding of eukaryotic H/ACA RNPs comes from structures of the human telomerase H/ACA RNP. Here we report a 2.7 Å cryo-electron microscopy structure of the telomerase H/ACA RNP. The significant improvement in resolution over previous 3.3 Å to 8.2 Å structures allows us to uncover new molecular interactions within the H/ACA RNP. Many disease mutations are mapped to these interaction sites. The structure also reveals unprecedented insights into a region critical for pseudouridylation in canonical H/ACA RNPs. Together, our work advances understanding of telomerase-related disease mutations and the mechanism of pseudouridylation by eukaryotic H/ACA RNPs.

PubMed: 38272871
DOI: 10.1038/s41467-024-45002-x

実験手法

ELECTRON MICROSCOPY (2.7 Å)