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 neurotransmission
2017
Drosophila CG3303 is an essential endoribonuclease linked to TDP-43-mediated neurodegeneration
Laneve P, Piacentini L, Casale AM, Capauto D, Gioia U, Cappucci U, Di Carlo V, Bozzoni I, Di Micco P, Morea V, Di Franco CA, Caffarelli E. Drosophila CG3303 is an essential endoribonuclease linked to TDP-43-mediated neurodegeneration. Scientific Reports 2017, 7: 41559. PMID: 28139767, PMCID: PMC5282483, DOI: 10.1038/srep41559.Peer-Reviewed Original ResearchConceptsEukaryotic RNA metabolismDrosophila nervous systemTDP-43-mediated neurodegenerationImportant biological processesNovel family memberUnique biochemical featuresEssential endoribonucleaseRibosome biogenesisDrosophila viabilityRNA metabolismPositive regulatorFunctional characterisationBiosynthetic enzymesRibonuclease familyGene productsBiological processesMolecular levelNervous system activityBiochemical featuresCholinergic circuitsPhenotypeViral replicationNervous systemImmature phenotypeFamily members