Polyglutamine disorders: Pathogenesis and potential drug interventions
Polyglutamine/poly (Q) diseases are a group nine hereditary neurodegenerative disorders
caused due to abnormally expanded stretches of CAG trinucleotide in functionally distinct …
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 …
functions in motor coordination, motor learning, cognition, and emotion. Such cerebellar …
Endosomal dysfunction contributes to cerebellar deficits in spinocerebellar ataxia type 6
Abstract Spinocerebellar ataxia type 6 (SCA6) is a rare disease that is characterized by
cerebellar dysfunction. Patients have progressive motor coordination impairment, and …
cerebellar dysfunction. Patients have progressive motor coordination impairment, and …
Mitochondrial damage and impaired mitophagy contribute to disease progression in SCA6
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 …
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 …
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 …
lacking. Here we show that constitutive knockout of two homologous genes encoding the …
Structured connectivity in the output of the cerebellar cortex
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 …
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 …
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) …
often attributed to cerebellar dysfunction and eventual degeneration of Purkinje cells (PCs) …