Both Ctrol and Kd organoids show strong myelin disruption at 28 weeks of myelination.
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posted on 2024-12-05, 18:56 authored by Lisa Marie P. Evans, Joseph Gawron, Fraser J. Sim, M. Laura Feltri, Leandro N. MarzialiImmunohistochemistry for MBP and Olig2 of Ctrol and Kd Organoids at 28 weeks of myelination.
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strong lamp1 reductionnormal culture conditionsmtor pathway dysregulationlater time pointcytotoxic lipid psychosineproduce myelinating organoidsgloboid cell formationderived myelinating organoidsupon galcer feedingovercome galcer accumulationlysosomal storage disorderbecome globoid cellsdigest myelin debrisautophagy protein lc3bderived microglia showkd microglia showgloboid cellskd organoidsgalcer ).accumulated galcerlysosomal galactosylceramidaselysosomal dysfunctionrecruits microgliavaluable toolsuggesting lackslight increasesimilar numbershorter internodesreverted showingreduced numberorganoid modelobvious defectsmyelin internodesmarginal ratekd patientskd ipschuman ipscearly eventscompensatory mechanismcompensatory capabilitiesautophagy proteins
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