Potential disease-modifying therapies for Huntington's disease: lessons learned and future opportunities

SJ Tabrizi, C Estevez-Fraga… - The Lancet …, 2022 - thelancet.com
Huntington's disease is the most frequent autosomal dominant neurodegenerative disorder;
however, no disease-modifying interventions are available for patients with this disease. The …

Astrocyte contribution to dysfunction, risk and progression in neurodegenerative disorders

AN Brandebura, A Paumier, TS Onur… - Nature Reviews …, 2023 - nature.com
There is increasing appreciation that non-neuronal cells contribute to the initiation,
progression and pathology of diverse neurodegenerative disorders. This Review focuses on …

Advancing cell therapy for neurodegenerative diseases

S Temple - Cell stem cell, 2023 - cell.com
Cell-based therapies are being developed for various neurodegenerative diseases that
affect the central nervous system (CNS). Concomitantly, the roles of individual cell types in …

Protein misfolding in neurodegenerative diseases: implications and strategies

P Sweeney, H Park, M Baumann, J Dunlop… - Translational …, 2017 - Springer
A hallmark of neurodegenerative proteinopathies is the formation of misfolded protein
aggregates that cause cellular toxicity and contribute to cellular proteostatic collapse …

Single-nucleus RNA-seq identifies Huntington disease astrocyte states

O Al-Dalahmah, AA Sosunov, A Shaik, K Ofori… - Acta neuropathologica …, 2020 - Springer
Huntington Disease (HD) is an inherited movement disorder caused by expanded CAG
repeats in the Huntingtin gene. We have used single nucleus RNASeq (snRNASeq) to …

Polyglutamine repeats in neurodegenerative diseases

AP Lieberman, VG Shakkottai… - Annual Review of …, 2019 - annualreviews.org
Among the age-dependent protein aggregation disorders, nine neurodegenerative diseases
are caused by expansions of CAG repeats encoding polyglutamine (polyQ) tracts. We …

SOX9 is an astrocyte-specific nuclear marker in the adult brain outside the neurogenic regions

W Sun, A Cornwell, J Li, S Peng, MJ Osorio… - Journal of …, 2017 - Soc Neuroscience
Astrocytes have in recent years become the focus of intense experimental interest, yet
markers for their definitive identification remain both scarce and imperfect. Astrocytes may …

Young glial progenitor cells competitively replace aged and diseased human glia in the adult chimeric mouse brain

R Vieira, JN Mariani, NPT Huynh… - Nature …, 2024 - nature.com
Competition among adult brain cells has not been extensively researched. To investigate
whether healthy glia can outcompete diseased human glia in the adult forebrain, we …

Astrocyte molecular signatures in Huntington's disease

B Diaz-Castro, MR Gangwani, X Yu… - Science translational …, 2019 - science.org
Astrocytes are implicated in neurodegenerative disorders and may contribute to striatal
neuron loss or dysfunction in Huntington's disease (HD). Here, we assessed striatal …

Conservation and divergence of vulnerability and responses to stressors between human and mouse astrocytes

J Li, L Pan, WG Pembroke, JE Rexach… - Nature …, 2021 - nature.com
Astrocytes play important roles in neurological disorders such as stroke, injury, and
neurodegeneration. Most knowledge on astrocyte biology is based on studies of mouse …