CatWalk XT gait parameters: a review of reported parameters in pre-clinical studies of multiple central nervous system and peripheral nervous system disease models
IK Timotius, RF Roelofs… - Frontiers in Behavioral …, 2023 - frontiersin.org
Automated gait assessment tests are used in studies of disorders characterized by gait
impairment. CatWalk XT is one of the first commercially available automated systems for …
impairment. CatWalk XT is one of the first commercially available automated systems for …
Dysregulation of zebrin-II cell subtypes in the cerebellum is a shared feature across polyglutamine ataxia mouse models and patients
LC Bartelt, PM Switonski, G Adamek, F Longo… - Science Translational …, 2024 - science.org
Spinocerebellar ataxia type 7 (SCA7) is a genetic neurodegenerative disorder caused by a
CAG-polyglutamine repeat expansion. Purkinje cells (PCs) are central to the pathology of …
CAG-polyglutamine repeat expansion. Purkinje cells (PCs) are central to the pathology of …
Acetylation-dependent SAGA complex dimerization promotes nucleosome acetylation and gene transcription
Cells reprogram their transcriptomes to adapt to external conditions. The SAGA (Spt-Ada-
Gcn5 acetyltransferase) complex is a highly conserved transcriptional coactivator that plays …
Gcn5 acetyltransferase) complex is a highly conserved transcriptional coactivator that plays …
Polyglutamine ataxias: our current molecular understanding and what the future holds for antisense therapies
Polyglutamine (polyQ) ataxias are a heterogenous group of neurological disorders all
caused by an expanded CAG trinucleotide repeat located in the coding region of each …
caused by an expanded CAG trinucleotide repeat located in the coding region of each …
Early molecular layer interneuron hyperactivity triggers Purkinje neuron degeneration in SCA1
F Pilotto, C Douthwaite, R Diab, XQ Ye, C Tietje… - Neuron, 2023 - cell.com
Toxic proteinaceous deposits and alterations in excitability and activity levels characterize
vulnerable neuronal populations in neurodegenerative diseases. Using in vivo two-photon …
vulnerable neuronal populations in neurodegenerative diseases. Using in vivo two-photon …
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 …
of the nervous system, in particular the cerebellum, and other systems that manifest with a …
Ogt deficiency induces abnormal cerebellar function and behavioral deficits of adult mice through modulating RhoA/ROCK signaling
J Zhang, K Wei, W Qu, M Wang, Q Zhu… - Journal of …, 2023 - Soc Neuroscience
Previous studies have shown the essential roles of O-GlcNAc transferase (Ogt) and O-
GlcNAcylation in neuronal development, function and neurologic diseases. However, the …
GlcNAcylation in neuronal development, function and neurologic diseases. However, the …
Longitudinal MRI and 1H-MRS study of SCA7 mouse forebrain reveals progressive multiregional atrophy and early brain metabolite changes indicating early …
JB Pérot, A Niewiadomska-Cimicka, E Brouillet… - Plos one, 2024 - journals.plos.org
SpinoCerebellar Ataxia type 7 (SCA7) is an inherited disorder caused by CAG triplet repeats
encoding polyglutamine expansion in the ATXN7 protein, which is part of the transcriptional …
encoding polyglutamine expansion in the ATXN7 protein, which is part of the transcriptional …
Polyglutamine-expanded ATXN7 alters a specific epigenetic signature underlying photoreceptor identity gene expression in SCA7 mouse retinopathy
A Niewiadomska-Cimicka, A Hache, S Le Gras… - Journal of Biomedical …, 2022 - Springer
Background Spinocerebellar ataxia type 7 (SCA7) is a neurodegenerative disorder that
primarily affects the cerebellum and retina. SCA7 is caused by a polyglutamine expansion in …
primarily affects the cerebellum and retina. SCA7 is caused by a polyglutamine expansion in …
Aberrant CHCHD2-associated mitochondriopathy in Kii ALS/PDC astrocytes
N Leventoux, S Morimoto, M Ishikawa… - Acta …, 2024 - Springer
Abstract Amyotrophic Lateral Sclerosis/Parkinsonism-Dementia Complex (ALS/PDC), a rare
and complex neurological disorder, is predominantly observed in the Western Pacific …
and complex neurological disorder, is predominantly observed in the Western Pacific …