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Bilateral striatal necrosis in a case with ADAR1-related Aicardi Goutières Syndrome.
Neurol Sci
January 2025
Division of Pediatric Neurology, Ankara University Faculty of Medicine, Ankara, Turkey.
The therapeutic effects of induced pluripotent stem cell-derived mesenchymal stem cells on Parkinson's disease.
IUBMB Life
January 2025
Cheerland Watson Precision Medicine Ltd, Shenzhen, China.
Parkinson's disease (PD), characterized by progressive degeneration of dopaminergic neurons in substantia nigra, has no disease-modifying therapy. Mesenchymal stem cell (MSC) therapy has shown great promise as a disease-modifying solution for PD. Induced pluripotent stem cell-derived MSC (iMSC) not only has stronger neural repair function, but also helps solve the problem of MSC heterogeneity.
View Article and Find Full Text PDFNeuropsychological profile of POLR3A-related spastic ataxia.
Neurol Sci
December 2024
Memory Clinic, Department of Neurology, Onze-Lieve-Vrouwziekenhuis, Aalst, Belgium.
Background And Objectives: POLR3-related disorders are a group of autosomal recessive neurodegenerative diseases that usually cause leukodystrophy and can lead to cognitive dysfunction. Literature reporting comprehensive neuropsychological assessment in POLR3A-related diseases is sparse. Here we describe the neuropsychological profile of a case of childhood-onset POLR3A-related spastic ataxia without leukodystrophy.
View Article and Find Full Text PDFIntracellular α-synuclein assemblies are sufficient to alter nanoscale diffusion in the striatal extracellular space.
NPJ Parkinsons Dis
December 2024
Univ. Bordeaux, CNRS, Institut des Maladies Neurodégénératives, UMR 5293, F-33000, Bordeaux, France.
α-synucleinopathies progression involves the spread of α-synuclein aggregates through the extracellular space (ECS). Single-particle tracking studies showed that α-synuclein-induced neurodegeneration increases ECS molecular diffusivity. To disentangle the consequences of neuronal loss versus α-synuclein-positive intracellular assemblies formation, we performed near-infrared single-particle tracking to characterise ECS rheology in the striatum of mouse models of α-synucleinopathies.
View Article and Find Full Text PDFSite-specific seeding of Lewy pathology induces distinct pre-motor cellular and dendritic vulnerabilities in the cortex.
Nat Commun
December 2024
Weldon School of Biomedical Engineering, West Lafayette, Indiana, IN, USA.
Circuit-based biomarkers distinguishing the gradual progression of Lewy pathology across synucleinopathies remain unknown. Here, we show that seeding of α-synuclein preformed fibrils in mouse dorsal striatum and motor cortex leads to distinct prodromal-phase cortical dysfunction across months. Our findings reveal that while both seeding sites had increased cortical pathology and hyperexcitability, distinct differences in electrophysiological and cellular ensemble patterns were crucial in distinguishing pathology spread between the two seeding sites.
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