Polyglutamine spinocerebellar ataxias: emerging therapeutic targets
A Neves-Carvalho, S Duarte-Silva… - Expert Opinion on …, 2020 - Taylor & Francis
Introduction Six of the most frequent dominantly inherited spinocerebellar ataxias (SCAs)
worldwide–SCA1, SCA2, SCA3, SCA6, SCA7, and SCA17–are caused by an expansion of a …
worldwide–SCA1, SCA2, SCA3, SCA6, SCA7, and SCA17–are caused by an expansion of a …
Oxidative Stress in Spinocerebellar Ataxia Type 3 and Its Attenuation by Herbal Remedies in Traditional Chinese Medicine: A Systematic Review
NS Mohd Hisam, KH Wong - Antioxidants, 2024 - mdpi.com
Spinocerebellar ataxia type 3 (SCA3) is an autosomal dominant neurodegenerative disorder
that gives rise to motor incoordination and progressive functional disabilities. Although …
that gives rise to motor incoordination and progressive functional disabilities. Although …
[HTML][HTML] Coaggregation of polyglutamine (polyQ) proteins is mediated by polyQ-tract interactions and impairs cellular proteostasis: Coaggregation of polyQ proteins
JY Hong, JY Wang, HW Yue, XL Zhang… - Acta Biochimica et …, 2023 - ncbi.nlm.nih.gov
Nine polyglutamine (polyQ) proteins have already been identified that are considered to be
associated with the pathologies of neurodegenerative disorders called polyQ diseases, but …
associated with the pathologies of neurodegenerative disorders called polyQ diseases, but …
Antibody-assisted selective isolation of Purkinje cell nuclei from mouse cerebellar tissue
LC Bartelt, M Fakhri, G Adamek, M Trybus… - Cell Reports …, 2024 - cell.com
We developed a method that utilizes fluorescent labeling of nuclear envelopes alongside
cytometry sorting for the selective isolation of Purkinje cell (PC) nuclei. Beginning with SUN1 …
cytometry sorting for the selective isolation of Purkinje cell (PC) nuclei. Beginning with SUN1 …
The S6k/4E-BP mediated growth promoting sub-pathway of insulin signalling cascade is essential to restrict pathogenesis of poly (Q) disorders in Drosophila
S Tandon, S Sarkar - Life Sciences, 2021 - Elsevier
Human neurodegenerative polyglutamine [poly (Q)] disorders, such as Huntington's disease
(HD) and spinocerebellar ataxias (SCA), are characterised by an abnormal expansion of …
(HD) and spinocerebellar ataxias (SCA), are characterised by an abnormal expansion of …
[HTML][HTML] Purkinje-Enriched snRNA-seq in SCA7 Cerebellum Reveals Zebrin Identity Loss as a Central Feature of Polyglutamine Ataxias
LC Bartelt, PM Switonski, G Adamek, J Carvalho… - bioRxiv, 2023 - ncbi.nlm.nih.gov
Abstract Spinocerebellar ataxia type 7 (SCA7) is an inherited neurodegenerative disorder
caused by a CAG-polyglutamine repeat expansion. SCA7 patients display a striking loss of …
caused by a CAG-polyglutamine repeat expansion. SCA7 patients display a striking loss of …
A comprehensive review of iPS cell line-based disease modelling of the polyglutamine spinocerebellar ataxias 2 and 3: a focus on the research outcomes
N Raghunathan, S Sankaran… - Annals of Medicine and …, 2024 - journals.lww.com
Spinocerebellar ataxias (SCAs) are a rare autosomal dominant neurodegenerative disorder.
To date, approximately 50 different subtypes of SCAs have been characterized. The …
To date, approximately 50 different subtypes of SCAs have been characterized. The …
[HTML][HTML] Gene editing as a therapeutic strategy for spinocerebellar ataxia type-3
N Déglon - Revue Neurologique, 2024 - Elsevier
Abstract Spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease, is
a neurodegenerative disease caused by expanded polyglutamine repeats in exon 10 of the …
a neurodegenerative disease caused by expanded polyglutamine repeats in exon 10 of the …
[HTML][HTML] Increased intrinsic membrane excitability is associated with hypertrophic olivary degeneration in spinocerebellar ataxia type 1
One of the characteristic areas of brainstem degeneration across multiple spinocerebellar
ataxias (SCAs) is the inferior olive (IO), a medullary nucleus that plays a key role in motor …
ataxias (SCAs) is the inferior olive (IO), a medullary nucleus that plays a key role in motor …
Spinocerebellar Ataxia Type 7: From Mechanistic Pathways to Therapeutic Opportunities
PM Switonski, AR La Spada - Trials for Cerebellar Ataxias: From Cellular …, 2023 - Springer
Abstract Spinocerebellar ataxia type 7 (SCA7) is a cerebellar and retinal neurodegenerative
disease caused by a CAG/polyglutamine (polyQ) expansion mutation in the ataxin-7 …
disease caused by a CAG/polyglutamine (polyQ) expansion mutation in the ataxin-7 …