[HTML][HTML] Purkinje cell signaling deficits in animal models of ataxia
Purkinje cell (PC) dysfunction or degeneration is the most frequent finding in animal models
with ataxic symptoms. Mutations affecting intrinsic membrane properties can lead to ataxia …
with ataxic symptoms. Mutations affecting intrinsic membrane properties can lead to ataxia …
[HTML][HTML] Aberrant cerebellar circuitry in the spinocerebellar ataxias
The spinocerebellar ataxias (SCAs) are a heterogeneous group of neurodegenerative
diseases that share convergent disease features. A common symptom of these diseases is …
diseases that share convergent disease features. A common symptom of these diseases is …
Activation of TrkB-Akt signaling rescues deficits in a mouse model of SCA6
Spinocerebellar ataxia type 6 (SCA6) is a neurodegenerative disease resulting in motor
coordination deficits and cerebellar pathology. Expression of brain-derived neurotrophic …
coordination deficits and cerebellar pathology. Expression of brain-derived neurotrophic …
[HTML][HTML] A mitochondrial-targeted antioxidant (MitoQ) improves motor coordination and reduces Purkinje cell death in a mouse model of ARSACS
Mitochondrial deficits have been observed in animal models of Autosomal-recessive spastic
ataxia of Charlevoix-Saguenay (ARSACS) and in patient-derived fibroblasts. We …
ataxia of Charlevoix-Saguenay (ARSACS) and in patient-derived fibroblasts. We …
[HTML][HTML] 4-aminopyridine reverses ataxia and cerebellar firing deficiency in a mouse model of spinocerebellar ataxia type 6
Abstract Spinocerebellar ataxia type 6 (SCA6) is a devastating midlife-onset autosomal
dominant motor control disease with no known treatment. Using a hyper-expanded …
dominant motor control disease with no known treatment. Using a hyper-expanded …
Inhibition of NLRP3 inflammasome by glibenclamide attenuated dopaminergic neurodegeneration and motor deficits in paraquat and maneb-induced mouse …
X Qiu, Q Wang, L Hou, C Zhang, Q Wang, X Zhao - Toxicology Letters, 2021 - Elsevier
Pesticides exposure can lead to damage of dopaminergic neurons, which are associated
with increased risk of Parkinson's disease (PD). However, the etiology of PD remains poorly …
with increased risk of Parkinson's disease (PD). However, the etiology of PD remains poorly …
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 …
[HTML][HTML] What is the pathogenic CAG expansion length in Huntington's disease?
J Donaldson, S Powell, N Rickards… - Journal of …, 2021 - content.iospress.com
Abstract Huntington's disease (HD)(OMIM 143100) is caused by an expanded CAG repeat
tract in the HTT gene. The inherited CAG length is known to expand further in somatic and …
tract in the HTT gene. The inherited CAG length is known to expand further in somatic and …
Tissue beads: tissue‐specific extracellular matrix microbeads to potentiate reprogrammed cell‐based therapy
Microbeads have been utilized as efficient cell culture carriers and injectable scaffolds for
cell transplantation. However, various polymers currently used to generate microbeads have …
cell transplantation. However, various polymers currently used to generate microbeads have …
Endosomal dysfunction contributes to cerebellar deficits in spinocerebellar ataxia type 6
Abstract Spinocerebellar ataxia type 6 (SCA6) is a rare disease that is characterized by
cerebellar dysfunction. Patients have progressive motor coordination impairment, and …
cerebellar dysfunction. Patients have progressive motor coordination impairment, and …