[HTML][HTML] Role and mechanism of ferroptosis in neurological diseases

M Ou, Y Jiang, Y Ji, Q Zhou, Z Du, H Zhu, Z Zhou - Molecular Metabolism, 2022 - Elsevier
Background Ferroptosis, as a new form of cell death, is different from other cell deaths such
as autophagy or senescence. Ferroptosis involves in the pathophysiological progress of …

Molecular pathophysiological mechanisms in Huntington's disease

A Jurcau - Biomedicines, 2022 - mdpi.com
Huntington's disease is an inherited neurodegenerative disease described 150 years ago
by George Huntington. The genetic defect was identified in 1993 to be an expanded CAG …

[HTML][HTML] Microglia and complement mediate early corticostriatal synapse loss and cognitive dysfunction in Huntington's disease

DK Wilton, K Mastro, MD Heller, FW Gergits… - Nature medicine, 2023 - nature.com
Huntington's disease (HD) is a devastating monogenic neurodegenerative disease
characterized by early, selective pathology in the basal ganglia despite the ubiquitous …

[HTML][HTML] Pathogenesis of SCA3 and implications for other polyglutamine diseases

HS McLoughlin, LR Moore, HL Paulson - Neurobiology of disease, 2020 - Elsevier
Tandem repeat diseases include the neurodegenerative disorders known as polyglutamine
(polyQ) diseases, caused by CAG repeat expansions in the coding regions of the respective …

Huntington disease oligodendrocyte maturation deficits revealed by single-nucleus RNAseq are rescued by thiamine-biotin supplementation

RG Lim, O Al-Dalahmah, J Wu, MP Gold… - Nature …, 2022 - nature.com
The complexity of affected brain regions and cell types is a challenge for Huntington's
disease (HD) treatment. Here we use single nucleus RNA sequencing to investigate …

[HTML][HTML] The contribution of glial cells to Huntington's disease pathogenesis

DK Wilton, B Stevens - Neurobiology of disease, 2020 - Elsevier
Glial cells play critical roles in the normal development and function of neural circuits, but in
many neurodegenerative diseases, they become dysregulated and may contribute to the …

A TCF7L2-responsive suppression of both homeostatic and compensatory remyelination in Huntington disease mice

A Benraiss, JN Mariani, A Tate, PM Madsen, KM Clark… - Cell Reports, 2022 - cell.com
Huntington's disease (HD) is characterized by defective oligodendroglial differentiation and
white matter disease. Here, we investigate the role of oligodendrocyte progenitor cell (OPC) …

Impaired oligodendrocyte maturation is an early feature in SCA3 disease pathogenesis

KH Schuster, AJ Zalon, H Zhang… - Journal of …, 2022 - Soc Neuroscience
Spinocerebellar ataxia Type 3 (SCA3), the most common dominantly inherited ataxia, is a
polyglutamine neurodegenerative disease for which there is no disease-modifying therapy …

BMAL1 loss in oligodendroglia contributes to abnormal myelination and sleep

D Rojo, L Dal Cengio, A Badner, S Kim, N Sakai… - Neuron, 2023 - cell.com
Myelination depends on the maintenance of oligodendrocytes that arise from
oligodendrocyte precursor cells (OPCs). We show that OPC-specific proliferation …

The translational potential of cholesterol-based therapies for neurological disease

M Valenza, G Birolini, E Cattaneo - Nature Reviews Neurology, 2023 - nature.com
Cholesterol is an important metabolite and membrane component and is enriched in the
brain owing to its role in neuronal maturation and function. In the adult brain, cholesterol is …