title: Functions of ribosomal proteins in assembly of eukaryotic ribosomes in vivo.
abstract: The proteome of cells is synthesized by ribosomes, complex ribonucleoproteins that in eukaryotes contain 79-80 proteins and four ribosomal RNAs (rRNAs) more than 5,400 nucleotides long. How these molecules assemble together and how their assembly is regulated in concert with the growth and proliferation of cells remain important unanswered questions. Here, we review recently emerging principles to understand how eukaryotic ribosomal proteins drive ribosome assembly in vivo. Most ribosomal proteins assemble with rRNA cotranscriptionally; their association with nascent particles is strengthened as assembly proceeds. Each subunit is assembled hierarchically by sequential stabilization of their subdomains. The active sites of both subunits are constructed last, perhaps to prevent premature engagement of immature ribosomes with active subunits. Late-assembly intermediates undergo quality-control checks for proper function. Mutations in ribosomal proteins that affect mostly late steps lead to ribosomopathies, diseases that include a spectrum of cell type-specific disorders that often transition from hypoproliferative to hyperproliferative growth.
authors.affiliation: Instituto de Biomedicina de Sevilla, Hospital Universitario Virgen del Rocio/CSIC/Universidad de Sevilla, E-41013 Sevilla, Spain.|Departamento de Genetica, Universidad de Sevilla, E-41013 Sevilla, Spain.
authors.forename: Jesus
authors.initials: J
authors.lastname: de la Cruz
authors.affiliation: Department of Cancer Biology, The Scripps Research Institute, Jupiter, Florida 33458.
authors.forename: Katrin
authors.initials: K
authors.lastname: Karbstein
authors.affiliation: Department of Biological Sciences, Carnegie Mellon University, Pittsburgh, Pennsylvania 15213.
authors.forename: John L
authors.initials: JL
authors.lastname: Woolford
mesh_terms: D000818:Animals; D005057:Eukaryotic Cells / Q000737:chemistry / Q000378:metabolism*; D006801:Humans; D008958:Models, Molecular; D012269:Ribosomal Proteins / Q000737:chemistry / Q000378:metabolism*; D012270:Ribosomes / Q000737:chemistry* / Q000378:metabolism*
chemical_list: D012269:Ribosomal Proteins
keywords: 40S ribosomal subunits; 60S ribosomal subunits; RNA–protein interactions; pre-rRNA processing; rRNA folding; ribosome assembly