The molecular basis for ANE syndrome revealed by the large ribosomal subunit processome interactome
McCann KL, Teramoto T, Zhang J, Hall T, Baserga SJ. The molecular basis for ANE syndrome revealed by the large ribosomal subunit processome interactome. ELife 2016, 5: e16381. PMID: 27077951, PMCID: PMC4859800, DOI: 10.7554/elife.16381.Peer-Reviewed Original ResearchMeSH KeywordsAlopeciaCircular DichroismEndocrine System DiseasesHumansIntellectual DisabilityMagnetic Resonance SpectroscopyModels, BiologicalMutant ProteinsProtein BindingProtein FoldingProtein Interaction MapsRibonucleoproteins, Small NucleolarRibosome Subunits, LargeRNA PrecursorsRNA Processing, Post-TranscriptionalRNA-Binding ProteinsSaccharomyces cerevisiaeSaccharomyces cerevisiae ProteinsConceptsProtein-protein interactionsANE syndromeMolecular basisDefective protein foldingRRNA processing defectsRNA recognition motifMature ribosomesRibosome assemblyHub proteinsRRNA processingNucleolar proteinsDomain foldingProtein foldingRecognition motifHuman diseasesProcessing defectsInteractomeMutationsCircular dichroismHub functionModel systemYeastFoldingProteinNOP4A nucleolar role for the Fanconi anemia pathway protein, FANCI, in rDNA transcription
Sondalle S, Longerich S, Sung P, Baserga S. A nucleolar role for the Fanconi anemia pathway protein, FANCI, in rDNA transcription. The FASEB Journal 2016, 30 DOI: 10.1096/fasebj.30.1_supplement.1048.1.Peer-Reviewed Original ResearchRNA polymerase IRibosome biogenesisDNA repairFanconi anemiaNucleolar proteinsNucleolar roleHeLa cell nuclear extractsPre-ribosomal RNANovel regulatory mechanismCell nuclear extractsDNA repair defectsInterstrand DNA crosslinksMature ribosomesRDNA transcriptionRRNA transcriptionLarge subunitNucleolar functionFA proteinsFANCIUbiquitination stateSubcellular fractionationReduced transcriptionPolymerase IBiogenesisRegulatory mechanismsWhen Good Ribosomes Go Bad
Baserga S, McCann K, Teramoto T, Zhang J, Tanaka Hall T. When Good Ribosomes Go Bad. The FASEB Journal 2016, 30 DOI: 10.1096/fasebj.30.1_supplement.387.1.Peer-Reviewed Original ResearchProtein-protein interactionsRNA recognition motifANE syndromeRibosome biogenesisRRNA processingProtein functionMolecular basisPre-rRNA processing defectThird RNA recognition motifDefective protein foldingHuman genetic diseasesFull-length proteinSingle amino acid substitutionNumerous human disordersAmino acid substitutionsYeast orthologMature ribosomesEukaryotic cellsGood ribosomeRibosome synthesisHub proteinsNucleolar proteinsNucleolar functionDomain foldingNucleolar dysfunction