Featured Publications
Fanconi anemia protein FANCI functions in ribosome biogenesis
Sondalle SB, Longerich S, Ogawa LM, Sung P, Baserga SJ. Fanconi anemia protein FANCI functions in ribosome biogenesis. Proceedings Of The National Academy Of Sciences Of The United States Of America 2019, 116: 2561-2570. PMID: 30692263, PMCID: PMC6377447, DOI: 10.1073/pnas.1811557116.Peer-Reviewed Original ResearchHuman diseases of the SSU processome
Sondalle SB, Baserga SJ. Human diseases of the SSU processome. Biochimica Et Biophysica Acta 2013, 1842: 758-764. PMID: 24240090, PMCID: PMC4058823, DOI: 10.1016/j.bbadis.2013.11.004.Peer-Reviewed Reviews, Practice Guidelines, Standards, and Consensus StatementsConceptsNorth American Indian childhood cirrhosisSSU processome componentsSSU processomeBowen-Conradi syndromeHuman diseasesSmall subunit processomeProduction of ribosomesRibosomal protein assemblyLarge ribonucleoproteinRibosome biogenesisFunctional ribosomesEukaryotic cellsProcessomeProtein assembliesCellular machinesRibosomesRNA cleavageProtein synthesisRibonucleoproteinEukaryotesCirhinAssemblyRRNAsRibosomopathiesBiogenesisThe Ribosome Biogenesis Factor Nol11 Is Required for Optimal rDNA Transcription and Craniofacial Development in Xenopus
Griffin JN, Sondalle SB, del Viso F, Baserga SJ, Khokha MK. The Ribosome Biogenesis Factor Nol11 Is Required for Optimal rDNA Transcription and Craniofacial Development in Xenopus. PLOS Genetics 2015, 11: e1005018. PMID: 25756904, PMCID: PMC4354908, DOI: 10.1371/journal.pgen.1005018.Peer-Reviewed Original ResearchConceptsCranial neural crestCraniofacial developmentRibosome biogenesisRibosome biogenesis proteinsRibosome biogenesis defectsProduction of ribosomesPre-rRNA transcriptionHuman tissue culture cellsMulticellular organismsTissue culture cellsBiogenesis proteinsRDNA transcriptionBiogenesis defectsCraniofacial cartilageRRNA transcriptionNucleolar proteinsP53 rescueNeural crestCell survivalTranscriptionSkeletal phenotypeCulture cellsCritical functionsRibosomopathiesBiogenesisRPSA, a candidate gene for isolated congenital asplenia, is required for pre-rRNA processing and spleen formation in Xenopus
Griffin JN, Sondalle SB, Robson A, Mis EK, Griffin G, Kulkarni SS, Deniz E, Baserga SJ, Khokha MK. RPSA, a candidate gene for isolated congenital asplenia, is required for pre-rRNA processing and spleen formation in Xenopus. Development 2018, 145: dev166181. PMID: 30337486, PMCID: PMC6215398, DOI: 10.1242/dev.166181.Peer-Reviewed Original ResearchConceptsPre-rRNA processingSmall ribosomal subunitCommon disease-associated mutationDisease-associated mutationsRpsA mRNARibosome biogenesisRibosome productionRibosome functionRibosomal subunitCandidate genesHuman mRNAsProtein componentsImpairs expressionSpleen developmentMolecular patterningRPSASpleen anlageMutationsXenopusGenesFirst animal modelUniversal requirementMRNAThe pre-rRNA processing factor DEF is rate limiting for the pathogenesis of MYCN-driven neuroblastoma
Tao T, Sondalle SB, Shi H, Zhu S, Perez-Atayde AR, Peng J, Baserga SJ, Look AT. The pre-rRNA processing factor DEF is rate limiting for the pathogenesis of MYCN-driven neuroblastoma. Oncogene 2017, 36: 3852-3867. PMID: 28263972, PMCID: PMC5501763, DOI: 10.1038/onc.2016.527.Peer-Reviewed Original ResearchConceptsDigestive organ expansion factorSmall ribosomal subunitPeripheral sympathetic nervous systemPre-ribosomal RNA processingTransgenic zebrafish modelOverexpression of MYCNNeuroblastoma cellsRibosome biogenesisSSU processomeNucleolar factorsRNA processingSympathetic nervous systemRibosomal subunitZebrafish modelHuman neuroblastoma cell lineTumor growth rateHuman neuroblastoma cellsNeuroblastoma cell linesNovel siteZebrafishCell linesNervous systemHuman neuroblastomaDisease penetranceNeuroblastoma
2023
Human nucleolar protein 7 (NOL7) is required for early pre-rRNA accumulation and pre-18S rRNA processing
McCool M, Bryant C, Huang H, Ogawa L, Farley-Barnes K, Sondalle S, Abriola L, Surovtseva Y, Baserga S. Human nucleolar protein 7 (NOL7) is required for early pre-rRNA accumulation and pre-18S rRNA processing. RNA Biology 2023, 20: 257-271. PMID: 37246770, PMCID: PMC10228412, DOI: 10.1080/15476286.2023.2217392.Peer-Reviewed Original ResearchConceptsPre-rRNA accumulationRibosome biogenesisNonessential roleEukaryotic ribosome biogenesisEssential cellular processesNucleolar stress responsePre-rRNA levelsRRNA processingLikely orthologCellular processesAssociated proteinsTumor suppressorStress responseHuman cellsProtein synthesisProtein 7Human counterpartBiogenesisYeastOrthologsHomologSubcomplexAccumulationRRNATranscription