The H/ACA RNP assembly factor SHQ1 functions as an RNA mimic

Hélène Walbott, Rosario Machado-Pinilla, Dominique Liger, Magali Blaud, Stéphane Réty, Petar N. Grozdanov, Kate Godin, Herman van Tilbeurgh, Gabriele Varani, U. Thomas Meier, Nicolas Leulliot

Research output: Contribution to journalArticle

26 Scopus citations


SHQ1 is an essential assembly factor for H/ACA ribonucleoproteins (RNPs) required for ribosome biogenesis, premRNA splicing, and telomere maintenance. SHQ1 binds dyskerin/NAP57, the catalytic subunit of human H/ACA RNPs, and this interaction is modulated by mutations causing X-linked dyskeratosis congenita. We report the crystal structure of the C-terminal domain of yeast SHQ1, Shq1p, and its complex with yeast dyskerin/NAP57, Cbf5p, lacking its catalytic domain. The C-terminal domain of Shq1p interacts with the RNA-binding domain of Cbf5p and, through structural mimicry, uses the RNA-protein-binding sites to achieve a specific protein-protein interface. We propose that Shq1p operates as a Cbf5p chaperone during RNP assembly by acting as an RNA placeholder, thereby preventing Cbf5p from nonspecific RNA binding before association with an H/ACA RNA and the other core RNP proteins.

Original languageEnglish (US)
Number of pages1
JournalGenes and Development
Issue number22
StatePublished - Jan 1 2011


  • H/ACA RNPs
  • Ribonucleoprotein assembly
  • Telomerase
  • X-linked dyskeratosis congenita

ASJC Scopus subject areas

  • Genetics
  • Developmental Biology

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    Walbott, H., Machado-Pinilla, R., Liger, D., Blaud, M., Réty, S., Grozdanov, P. N., Godin, K., van Tilbeurgh, H., Varani, G., Thomas Meier, U., & Leulliot, N. (2011). The H/ACA RNP assembly factor SHQ1 functions as an RNA mimic. Genes and Development, 25(22).