2023
FUS Alters circRNA Metabolism in Human Motor Neurons Carrying the ALS-Linked P525L Mutation
Colantoni A, Capauto D, Alfano V, D’Ambra E, D’Uva S, Tartaglia G, Morlando M. FUS Alters circRNA Metabolism in Human Motor Neurons Carrying the ALS-Linked P525L Mutation. International Journal Of Molecular Sciences 2023, 24: 3181. PMID: 36834591, PMCID: PMC9968238, DOI: 10.3390/ijms24043181.Peer-Reviewed Original ResearchMeSH KeywordsAmyotrophic Lateral SclerosisHumansMicroRNAsMotor NeuronsMutationRNA, CircularRNA-Binding Protein FUSConceptsRNA metabolismHuman motor neuronsNuclear/cytoplasmic partitioningNon-canonical splicing eventsAspects of RNASubset of circRNAsCytoplasmic partitioningCytoplasmic circRNAsSplicing eventsMutant proteinsCircular RNAsMotor neuronsAlu repeatsExon compositionCircRNAsProteinRNAMutationsFUS mutationsKey eventsFUSPreferential bindingP525L mutationMetabolismPivotal role
2022
A multifunctional locus controls motor neuron differentiation through short and long noncoding RNAs
Carvelli A, Setti A, Desideri F, Galfrè SG, Biscarini S, Santini T, Colantoni A, Peruzzi G, Marzi MJ, Capauto D, Di Angelantonio S, Ballarino M, Nicassio F, Laneve P, Bozzoni I. A multifunctional locus controls motor neuron differentiation through short and long noncoding RNAs. The EMBO Journal 2022, 41: embj2021108918. PMID: 35698802, PMCID: PMC9251839, DOI: 10.15252/embj.2021108918.Peer-Reviewed Original ResearchConceptsMN differentiationPost-transcriptional roleSingle-cell sequencingMotor neuron differentiationPostmitotic motor neuronsTranscriptional unitsMESC differentiationTranscription factorsCRISPR/Transcriptional levelMiR-466iNeuron differentiationNeuronal differentiationFunctional relevanceMotor neuronsMiR-384-5pDifferentiationSeries of eventsProliferation-related factorsAdditional layerMutantsMiRNAsLncRNAsLociRNA
2018
A Regulatory Circuitry Between Gria2, miR-409, and miR-495 Is Affected by ALS FUS Mutation in ESC-Derived Motor Neurons
Capauto D, Colantoni A, Lu L, Santini T, Peruzzi G, Biscarini S, Morlando M, Shneider NA, Caffarelli E, Laneve P, Bozzoni I. A Regulatory Circuitry Between Gria2, miR-409, and miR-495 Is Affected by ALS FUS Mutation in ESC-Derived Motor Neurons. Molecular Neurobiology 2018, 55: 7635-7651. PMID: 29430619, PMCID: PMC6132778, DOI: 10.1007/s12035-018-0884-4.Peer-Reviewed Original ResearchConceptsAmyotrophic lateral sclerosisMotor neuronsFUS mutationsMouse embryonic stem cellsPathogenesis of ALSMiRNA-dependent regulationCorresponding mRNA targetsNeuron-specific RNATypes of RNAEmbryonic stem cellsCross talkGlutamate α-aminoIsoxazole propionic acidDisease-associated mutationsMolecular cross talkDisturbance of Ca2Juvenile-onset formMiRNA repressionRegulatory circuitryTranscriptome profilingMRNA targetsMultifunctional proteinLong RNAsMN degenerationExcitatory neurotransmissionCharacterization of the lncRNA transcriptome in mESC-derived motor neurons: Implications for FUS-ALS
Biscarini S, Capauto D, Peruzzi G, Lu L, Colantoni A, Santini T, Shneider NA, Caffarelli E, Laneve P, Bozzoni I. Characterization of the lncRNA transcriptome in mESC-derived motor neurons: Implications for FUS-ALS. Stem Cell Research 2018, 27: 172-179. PMID: 29449089, DOI: 10.1016/j.scr.2018.01.037.Peer-Reviewed Original ResearchMeSH KeywordsAmyotrophic Lateral SclerosisAnimalsCell DifferentiationHumansMiceMotor NeuronsRNA, Long NoncodingTranscriptomeConceptsLong non-coding transcriptomeMouse motor neuronsAmyotrophic lateral sclerosisNon-coding transcriptomeMotor neuronsSevere amyotrophic lateral sclerosisAberrant RNA metabolismLong non-coding RNAsStem cellsEmbryonic stem cellsNon-coding RNAsInduced pluripotent stem cellsNervous system developmentPluripotent stem cellsRNA metabolismLncRNA transcriptomeDifferentiation systemLateral sclerosisTDP-43TranscriptomeFUS-ALSCandidate lncRNAsCrucial playersCausative mutationsEssential role
2017
FUS affects circular RNA expression in murine embryonic stem cell-derived motor neurons
Errichelli L, Dini Modigliani S, Laneve P, Colantoni A, Legnini I, Capauto D, Rosa A, De Santis R, Scarfò R, Peruzzi G, Lu L, Caffarelli E, Shneider NA, Morlando M, Bozzoni I. FUS affects circular RNA expression in murine embryonic stem cell-derived motor neurons. Nature Communications 2017, 8: 14741. PMID: 28358055, PMCID: PMC5379105, DOI: 10.1038/ncomms14741.Peer-Reviewed Original ResearchConceptsMouse motor neuronsCircRNA biogenesisRNA-binding protein FUSEmbryonic stem cell-derived motor neuronsCircular RNA productionRNA biosynthetic processesCircular RNA expressionStem cell-derived motor neuronsCell-derived motor neuronsAmyotrophic lateral sclerosisMost circRNAsFUS mutantsSplicing junctionsProtein FUSSplicing reactionBiosynthetic processesRNA productionToxic gainCircRNAsMotor neuronsBiogenesisRNA expressionFunction activityNuclear levelsFUS