Extracellular matrix regulation in physiology and in brain disease

A Soles, A Selimovic, K Sbrocco, F Ghannoum… - International journal of …, 2023 - mdpi.com
The extracellular matrix (ECM) surrounds cells in the brain, providing structural and
functional support. Emerging studies demonstrate that the ECM plays important roles during …

The extra-cerebellar effects of spinocerebellar ataxia type 1 (SCA1): looking beyond the cerebellum

V Olmos, N Gogia, K Luttik, F Haidery, J Lim - Cellular and Molecular Life …, 2022 - Springer
Abstract Spinocerebellar ataxia type 1 (SCA1) is one of nine polyglutamine (polyQ) diseases
and is characterized as an adult late-onset, progressive, dominantly inherited genetic …

Disruption of the ATXN1-CIC complex reveals the role of additional nuclear ATXN1 interactors in spinocerebellar ataxia type 1

SL Coffin, MA Durham, L Nitschke, E Xhako, AM Brown… - Neuron, 2023 - cell.com
Summary Spinocerebellar ataxia type 1 (SCA1) is a paradigmatic neurodegenerative
disease in that it is caused by a mutation in a broadly expressed protein, ATXN1; however …

Decreasing mutant ATXN1 nuclear localization improves a spectrum of SCA1-like phenotypes and brain region transcriptomic profiles

HP Handler, L Duvick, JS Mitchell, M Cvetanovic… - Neuron, 2023 - cell.com
Summary Spinocerebellar ataxia type 1 (SCA1) is a dominant trinucleotide repeat
neurodegenerative disease characterized by motor dysfunction, cognitive impairment, and …

A chlorzoxazone–folic acid combination improves cognitive affective decline in SCA2-58Q mice

KS Marinina, IB Bezprozvanny, PA Egorova - Scientific reports, 2023 - nature.com
Abstract Spinocerebellar ataxia type 2 (SCA2) is a polyglutamine disorder caused by a
pathological expansion of CAG repeats in ATXN2 gene. SCA2 is accompanied by cerebellar …

Consensus paper: strengths and weaknesses of animal models of spinocerebellar ataxias and their clinical implications

J Cendelin, M Cvetanovic, M Gandelman, H Hirai… - The Cerebellum, 2022 - Springer
Spinocerebellar ataxias (SCAs) represent a large group of hereditary degenerative diseases
of the nervous system, in particular the cerebellum, and other systems that manifest with a …

Cerebellar contribution to threat probability in a SCA6 mouse model

P Bohne, M Rybarski, DBE Mourabit… - Human molecular …, 2022 - academic.oup.com
Fear and anxiety have proven to be essential during the evolutionary process. However, the
mechanisms involved in recognizing and categorizing threat probability (ie low to high) to …

[HTML][HTML] Chronic optogenetic stimulation of Bergman glia leads to dysfunction of EAAT1 and Purkinje cell death, mimicking the events caused by expression of …

AN Shuvaev, OS Belozor, O Mozhei… - Neurobiology of …, 2021 - Elsevier
Bergmann glia (BG) are highly specialized radial astrocytes of the cerebellar cortex, which
play a key role in the uptake of synaptic glutamate via the excitatory amino acid transporter …

Cognitive decline and mood alterations in the mouse model of spinocerebellar ataxia type 2

KS Marinina, IB Bezprozvanny, PA Egorova - The Cerebellum, 2024 - Springer
Abstract Spinocerebellar ataxia type 2 (SCA2) is a hereditary disorder, caused by an
expansion of polyglutamine in the ataxin-2 protein. Although the mutant protein is expressed …

Mood alterations in mouse models of Spinocerebellar Ataxia type 1

M Asher, JG Rosa, M Cvetanovic - Scientific reports, 2021 - nature.com
Abstract Spinocerebellar ataxia type 1 (SCA1) is a fatal neurodegenerative disease caused
by abnormal expansion of glutamine-encoding CAG repeats in the Ataxin-1 (ATXN1) gene …