Polyglutamine disorders: Pathogenesis and potential drug interventions

S Tandon, P Aggarwal, S Sarkar - Life Sciences, 2024 - Elsevier
Polyglutamine/poly (Q) diseases are a group nine hereditary neurodegenerative disorders
caused due to abnormally expanded stretches of CAG trinucleotide in functionally distinct …

Regulation of cerebellar network development by granule cells and their molecules

M Kim, S Jun, H Park, K Tanaka-Yamamoto… - Frontiers in Molecular …, 2023 - frontiersin.org
The well-organized cerebellar structures and neuronal networks are likely crucial for their
functions in motor coordination, motor learning, cognition, and emotion. Such cerebellar …

Endosomal dysfunction contributes to cerebellar deficits in spinocerebellar ataxia type 6

AA Cook, TCS Leung, M Rice, M Nachman… - Elife, 2023 - elifesciences.org
Abstract Spinocerebellar ataxia type 6 (SCA6) is a rare disease that is characterized by
cerebellar dysfunction. Patients have progressive motor coordination impairment, and …

Mitochondrial damage and impaired mitophagy contribute to disease progression in SCA6

TCS Leung, E Fields, N Rana, RYL Shen… - Acta …, 2024 - Springer
Abstract Spinocerebellar ataxia type 6 (SCA6) is a neurodegenerative disease that
manifests in midlife and progressively worsens with age. SCA6 is rare, and many patients …

Targeting ion channels and Purkinje neuron intrinsic membrane excitability as a therapeutic strategy for cerebellar ataxia

H Huang, VG Shakkottai - Life, 2023 - mdpi.com
In degenerative neurological disorders such as Parkinson's disease, a convergence of
widely varying insults results in a loss of dopaminergic neurons and, thus, the motor …

Activation of TrkB signaling mitigates cerebellar anomalies caused by Rbm4-Bdnf deficiency

YY Tsai, CL Shen, CY Tsai, WY Tarn - Communications Biology, 2023 - nature.com
A molecular and functional link between neurotrophin signaling and cerebellar foliation is
lacking. Here we show that constitutive knockout of two homologous genes encoding the …

Structured connectivity in the output of the cerebellar cortex

KM Gruver, JWY Jiao, E Fields, S Song… - Nature …, 2024 - nature.com
The spatial organization of a neuronal circuit is critically important for its function since the
location of neurons is often associated with function. In the cerebellum, the major output of …

The Role of Protein Quantity Control in Polyglutamine Spinocerebellar Ataxias

H Zhang, X Wang - The Cerebellum, 2024 - Springer
Polyglutamine spinocerebellar ataxias (polyQ SCAs) represent the most prevalent subtype
of SCAs. The primary pathogenic mechanism is believed to be the gain-of-function …

[HTML][HTML] Ablation of TrkB from Enkephalinergic Precursor-Derived Cerebellar Granule Cells Generates Ataxia

E Eliseeva, MY Malik, L Minichiello - Biology, 2024 - mdpi.com
Simple Summary Patients suffering from ataxia disorders show symptoms of incoordination
often attributed to cerebellar dysfunction and eventual degeneration of Purkinje cells (PCs) …