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 …

Advances in cerebellar disorders: pre-clinical models, therapeutic targets, and challenges

M Manto, J Cendelin, M Strupp… - Expert Opinion on …, 2023 - Taylor & Francis
ABSTRACT Introduction Cerebellar ataxias (CAs) represent neurological disorders with
multiple etiologies and a high phenotypic variability. Despite progress in the understanding …

[HTML][HTML] Different Purkinje cell pathologies cause specific patterns of progressive gait ataxia in mice

D Jaarsma, MB Birkisdóttir, R van Vossen… - Neurobiology of …, 2024 - Elsevier
Gait ataxia is one of the most common and impactful consequences of cerebellar
dysfunction. Purkinje cells, the sole output neurons of the cerebellar cortex, are often …

Combinational treatments of RNA interference and extracellular vesicles in the spinocerebellar ataxia

Y Ding, Y Zhang, X Liu - Frontiers in Molecular Neuroscience, 2022 - frontiersin.org
Spinocerebellar ataxia (SCA) is an autosomal dominant neurodegenerative disease (ND)
with a high mortality rate. Symptomatic treatment is the only clinically adopted treatment …

Optogenetic and chemogenetic approaches for modeling neurological disorders in vivo

VG Krut, AL Kalinichenko, DI Maltsev, D Jappy… - Progress in …, 2024 - Elsevier
Animal models of human neurological disorders provide valuable experimental tools which
enable us to study various aspects of disorder pathogeneses, ranging from structural …

Viral-based animal models in polyglutamine disorders

C Henriques, MM Lopes, AC Silva, DD Lobo, RA Badin… - Brain, 2024 - academic.oup.com
Polyglutamine disorders are a complex group of incurable neurodegenerative disorders
caused by an abnormal expansion in the trinucleotide cytosine-adenine-guanine tract of the …

Psychiatric-like impairments in mouse models of spinocerebellar ataxias

F Tichanek - The Cerebellum, 2023 - Springer
Many patients with spinocerebellar ataxia (SCA) suffer from diverse neuropsychiatric issues,
including memory impairments, apathy, depression, or anxiety. These neuropsychiatric …

An insight into the iPSCs-derived two-dimensional culture and three-dimensional organoid models for neurodegenerative disorders

A Bhargava, AM Sandoval Castellanos… - Interface …, 2022 - royalsocietypublishing.org
The use of induced pluripotent stem cells (iPSCs) is a promising approach when used as
models to study neurodegenerative disorders (NDDs) in vitro. iPSCs have been used in in …

Slc9a6 mutation causes Purkinje cell loss and ataxia in the shaker rat

KP Figueroa, CJ Anderson, S Paul… - Human Molecular …, 2023 - academic.oup.com
The shaker rat carries a naturally occurring mutation leading to progressive ataxia
characterized by Purkinje cell (PC) loss. We previously reported on fine-mapping the shaker …

Reduction of Microvessel Number and Length in the Cerebellum of Purkinje Cell Degeneration Mice

Y Kolinko, M Kralickova, J Cendelin - The Cerebellum, 2024 - Springer
Degenerative effects of nerve tissues are often accompanied by changes in vascularization.
In this regard, knowledge about hereditary cerebellar degeneration is limited. In this study …