Corrigendum to “Cell Death Mechanisms in a Mouse Model of Retinal Degeneration in Spinocerebellar Ataxia 7” [Neuroscience 400C (2019) 72–84]
نویسندگان
چکیده
منابع مشابه
Spinocerebellar ataxia type 7 without retinal degeneration: a case report.
A 60-yr-old man developed progressive gait disturbance and limb ataxia at the age of 52. Family history was absent for neurological disorders. Examinations showed pure cerebellar syndrome. There was no retinal degeneration for 7 yr. A brain MRI done at the age of 56 showed atrophy of the cerebellar hemispheres and vermis. Genetic test confirmed the spinocerebellar ataxia type 7 with CAG repeat ...
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Autosomal dominant cerebellar ataxia (ADCA) was classified into Type I, Type II, and Type III, based on the clinical phenotypes by Harding. ADCA Type I presents with both cerebellar and noncerebellar signs and includes SCA1–SCA4, SCA8, SCA10, SCA12-SCA23, SCA25, SCA27, SCA28, and SCA32–SCA36. ADCA Type II consists of syndromes in association with pigmentary retinopathies and includes SCA7. ADCA...
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Autosomal dominant spinocerebellar ataxias (SCAs) are a clinical and molecular heterogeneous group of neurodegenerative disorders caused by expansion of an unstable CAG (polyglutamine) trinucleotide repeat. The cloning of the genes responsible and the characterization of the mutation now permit a reliable diagnosis. A wide spectrum of clinical phenotypes has been observed within families with t...
متن کاملRNA Interference-Based Therapy for Spinocerebellar Ataxia Type 7 Retinal Degeneration
Spinocerebellar ataxia type 7 (SCA7) is an autosomal dominant neurodegenerative disease characterized by loss of motor coordination and retinal degeneration with no current therapies in the clinic. The causative mutation is an expanded CAG repeat in the ataxin-7 gene whose mutant protein product causes cerebellar and brainstem degeneration and retinal cone-rod dystrophy. Here, we reduced the ex...
متن کاملPolyglutamine toxicity induces rod photoreceptor division, morphological transformation or death in spinocerebellar ataxia 7 mouse retina.
In neurodegenerative disorders caused by polyglutamine (polyQ) expansion, polyQ toxicity is thought to trigger a linear cascade of successive degenerative events leading to neuronal death. To understand how neurons cope with polyQ toxicity, we studied a Spinocerebellar ataxia 7 (SCA7) mouse which expresses polyQ-expanded ATXN7 only in rod photoreceptors. We show that in response to polyQ toxici...
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ژورنال
عنوان ژورنال: Neuroscience
سال: 2019
ISSN: 0306-4522
DOI: 10.1016/j.neuroscience.2019.01.058