title: The H/ACA RNP assembly factor SHQ1 functions as an RNA mimic.
abstract: SHQ1 is an essential assembly factor for H/ACA ribonucleoproteins (RNPs) required for ribosome biogenesis, pre-mRNA 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.
authors.affiliation: Institut de Biochimie et de Biophysique Moléculaire et Cellulaire, Université de Paris-Sud, Orsay Cedex, France.
authors.forename: Hélène
authors.initials: H
authors.lastname: Walbott
authors.forename: Rosario
authors.initials: R
authors.lastname: Machado-Pinilla
authors.forename: Dominique
authors.initials: D
authors.lastname: Liger
authors.forename: Magali
authors.initials: M
authors.lastname: Blaud
authors.forename: Stéphane
authors.initials: S
authors.lastname: Réty
authors.forename: Petar N
authors.initials: PN
authors.lastname: Grozdanov
authors.forename: Kate
authors.initials: K
authors.lastname: Godin
authors.forename: Herman
authors.initials: H
authors.lastname: van Tilbeurgh
authors.forename: Gabriele
authors.initials: G
authors.lastname: Varani
authors.forename: U Thomas
authors.initials: UT
authors.lastname: Meier
authors.forename: Nicolas
authors.initials: N
authors.lastname: Leulliot
mesh_terms: D002470:Cell Survival; D006801:Humans; D006836:Hydro-Lyases / Q000737:chemistry / Q000378:metabolism; D008869:Microtubule-Associated Proteins / Q000737:chemistry / Q000378:metabolism; D008958:Models, Molecular; D018716:Molecular Mimicry; D009154:Mutation; D009687:Nuclear Proteins / Q000737:chemistry* / Q000235:genetics / Q000378:metabolism*; D011485:Protein Binding; D017510:Protein Folding; D017434:Protein Structure, Tertiary; D012331:RNA, Fungal / Q000378:metabolism; D011994:Recombinant Proteins / Q000378:metabolism; D017411:Ribonucleoproteins, Small Nuclear / Q000737:chemistry / Q000378:metabolism; D020718:Ribonucleoproteins, Small Nucleolar / Q000737:chemistry / Q000378:metabolism*; D012441:Saccharomyces cerevisiae / Q000737:chemistry / Q000235:genetics / Q000378:metabolism; D029701:Saccharomyces cerevisiae Proteins / Q000737:chemistry* / Q000235:genetics / Q000378:metabolism*
chemical_list: D008869:Microtubule-Associated Proteins; C107170:NAP57; D009687:Nuclear Proteins; D012331:RNA, Fungal; D011994:Recombinant Proteins; D017411:Ribonucleoproteins, Small Nuclear; D020718:Ribonucleoproteins, Small Nucleolar; D029701:Saccharomyces cerevisiae Proteins; C470008:Shq1 protein, S cerevisiae; D006836:Hydro-Lyases; C082278:CBF5 protein, S cerevisiae
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