[HTML][HTML] Partial loss of ataxin-1 function contributes to transcriptional dysregulation in spinocerebellar ataxia type 1 pathogenesis
Spinocerebellar ataxia type 1 (SCA1) is a dominantly inherited neurodegenerative disease
caused by expansion of a CAG repeat that encodes a polyglutamine tract in ATAXIN1 …
caused by expansion of a CAG repeat that encodes a polyglutamine tract in ATAXIN1 …
[HTML][HTML] Mutant ataxin1 disrupts cerebellar development in spinocerebellar ataxia type 1
Spinocerebellar ataxia type 1 (SCA1) is an adult-onset neurodegenerative disease caused
by a polyglutamine expansion in the protein ATXN1, which is involved in transcriptional …
by a polyglutamine expansion in the protein ATXN1, which is involved in transcriptional …
ATXN2‐AS, a gene antisense to ATXN2, is associated with spinocerebellar ataxia type 2 and amyotrophic lateral sclerosis
Objective Spinocerebellar ataxia type 2 (SCA2) is a neurodegenerative disease caused by a
CAG repeat expansion in the gene ataxin‐2 (ATXN2). ATXN2 intermediate‐length CAG …
CAG repeat expansion in the gene ataxin‐2 (ATXN2). ATXN2 intermediate‐length CAG …
Comparison of spinocerebellar ataxia type 3 mouse models identifies early gain-of-function, cell-autonomous transcriptional changes in oligodendrocytes
Abstract Spinocerebellar ataxia type 3 (SCA3) is a neurodegenerative disorder caused by a
polyglutamine-encoding CAG repeat expansion in the ATXN3 gene. This expansion leads to …
polyglutamine-encoding CAG repeat expansion in the ATXN3 gene. This expansion leads to …
[HTML][HTML] A new humanized ataxin-3 knock-in mouse model combines the genetic features, pathogenesis of neurons and glia and late disease onset of SCA3/MJD
PM Switonski, WJ Szlachcic, WJ Krzyzosiak… - Neurobiology of …, 2015 - Elsevier
Abstract Spinocerebellar ataxia type 3 (SCA3/MJD) is a neurodegenerative disease
triggered by the expansion of CAG repeats in the ATXN3 gene. Here, we report the …
triggered by the expansion of CAG repeats in the ATXN3 gene. Here, we report the …
Early activation of microglia and astrocytes in mouse models of spinocerebellar ataxia type 1
M Cvetanovic, M Ingram, H Orr, P Opal - Neuroscience, 2015 - Elsevier
Abstract Spinocerebellar ataxia type 1 (SCA1) is an incurable, dominantly inherited
neurodegenerative disease of the cerebellum caused by a polyglutamine-repeat expansion …
neurodegenerative disease of the cerebellum caused by a polyglutamine-repeat expansion …
[HTML][HTML] Mouse ataxin-3 functional knock-out model
PM Switonski, A Fiszer, K Kazmierska, M Kurpisz… - Neuromolecular …, 2011 - Springer
Abstract Spinocerebellar ataxia 3 (SCA3) is a genetic disorder resulting from the expansion
of the CAG repeats in the ATXN3 gene. The pathogenesis of SCA3 is based on the toxic …
of the CAG repeats in the ATXN3 gene. The pathogenesis of SCA3 is based on the toxic …
[HTML][HTML] dAtaxin-2 Mediates Expanded Ataxin-1-Induced Neurodegeneration in a Drosophila Model of SCA1
I Al-Ramahi, AM Pérez, J Lim, M Zhang… - PLoS …, 2007 - journals.plos.org
Spinocerebellar ataxias (SCAs) are a genetically heterogeneous group of
neurodegenerative disorders sharing atrophy of the cerebellum as a common feature. SCA1 …
neurodegenerative disorders sharing atrophy of the cerebellum as a common feature. SCA1 …
[HTML][HTML] Loss of the Spinocerebellar Ataxia type 3 disease protein ATXN3 alters transcription of multiple signal transduction pathways
Spinocerebellar ataxia type 3 (SCA3) is a dominantly inherited neurodegenerative disorder
caused by a polyglutamine-encoding CAG repeat expansion in the ATXN3 gene which …
caused by a polyglutamine-encoding CAG repeat expansion in the ATXN3 gene which …
Impaired oligodendrocyte maturation is an early feature in SCA3 disease pathogenesis
Spinocerebellar ataxia Type 3 (SCA3), the most common dominantly inherited ataxia, is a
polyglutamine neurodegenerative disease for which there is no disease-modifying therapy …
polyglutamine neurodegenerative disease for which there is no disease-modifying therapy …