Movement disorders : official journal of the Movement Disorder Society · 2025
Oligodendrocyte Inclusion Pathology in Fragile X-Associated Tremor/Ataxia Syndrome.
СтатьяНазвание и аннотация: Перевод в очереди
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McLennan Yingratana, Aliashrafzadeh Hassan, Zirkelbach-Ngai Faith, McKenzie Forrest, Dufour Brett D, Becerra-Hernández Lina V, Escobar Adolfo Sanchez, Tassone Flora, Hagerman Paul, Hagerman Randi, Martínez-Cerdeño Veronica
Аннотация
BACKGROUND: Fragile X-associated tremor/ataxia syndrome (FXTAS) is a neurodegenerative disorder marked by white matter degeneration and intranuclear inclusions. While astrocytic and neuronal inclusions are well-documented, oligodendrocytes were previously thought to lack such pathology.
OBJECTIVE: To demonstrate that oligodendrocytes in the prefrontal cortex of FXTAS patients do harbor intranuclear inclusions, with significantly higher burden in white matter than gray matter.
METHODS: Ubiquitin and p62 immunofluorescence and enzymatic staining were employed to confirm the presence of intranuclear inclusions in oligodendrocytes across multiple brain regions.
RESULTS: Oligodendrocytes contain inclusions and inclusion burden is correlated with FMR1 CGG repeat length (ρ = 0.97, P < 0.001) in white matter.
CONCLUSIONS: These findings implicate oligodendrocyte dysfunction in FXTAS pathogenesis which may contribute to demyelination and white matter degeneration. Our data emphasize the need to consider cell type-specific mechanisms in FXTAS and support future therapeutic efforts aimed at restoring glial proteostasis. © 2025 International Parkinson and Movement Disorder Society.
OBJECTIVE: To demonstrate that oligodendrocytes in the prefrontal cortex of FXTAS patients do harbor intranuclear inclusions, with significantly higher burden in white matter than gray matter.
METHODS: Ubiquitin and p62 immunofluorescence and enzymatic staining were employed to confirm the presence of intranuclear inclusions in oligodendrocytes across multiple brain regions.
RESULTS: Oligodendrocytes contain inclusions and inclusion burden is correlated with FMR1 CGG repeat length (ρ = 0.97, P < 0.001) in white matter.
CONCLUSIONS: These findings implicate oligodendrocyte dysfunction in FXTAS pathogenesis which may contribute to demyelination and white matter degeneration. Our data emphasize the need to consider cell type-specific mechanisms in FXTAS and support future therapeutic efforts aimed at restoring glial proteostasis. © 2025 International Parkinson and Movement Disorder Society.