Polyglutamine inclusion body toxicity
نویسندگان
چکیده
منابع مشابه
Generation of neuronal intranuclear inclusions by polyglutamine-GFP: analysis of inclusion clearance and toxicity as a function of polyglutamine length.
Recent evidence suggests that, in huntingtin and many other proteins, polyglutamine repeats are a toxic stimulus in neurodegenerative diseases. To investigate the mechanism by which these repeats may be toxic, we transfected primary rat cerebellar granule neurons with polyglutamine-green fluorescent protein (GFP) fusion constructs containing 19 (Q19-GFP), 35 (Q35-GFP), 56 (Q56-GFP), or 80 (Q80-...
متن کاملSoluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic.
Expanded polyglutamine (polyQ) repeats cause neurodegenerative disorders, but their cytotoxic structures remain to be elucidated. Although soluble polyQ oligomers have been proposed as a cytotoxic structure, the cytotoxicity of soluble polyQ oligomers, not inclusion bodies (IBs), has not been proven in living cells. To clarify the cytotoxicity of soluble polyQ oligomers, we carried our fluoresc...
متن کاملStudies of polyglutamine expanded Ataxin-7 toxicity
Spinocerebellar ataxia type 7 (SCA7) is an autosomal dominant inherited neurodegenerative disease for which there is no cure. SCA7 belongs to the group of polyglutamine disorders, which are all caused by the expansion of a polyglutamine tract in different disease proteins. Common toxic mechanisms have been proposed for polyglutamine diseases; however the exact pathological mechanism(s) are stil...
متن کاملMechanisms of Polyglutamine Expanded Huntingtin Induced Toxicity
2003 ii We have read this dissertation entitled " Mechanisms of Polyglutamine Expanded Huntingtin Induced Toxicity " by Haibing Jiang, and recommend that it be accepted towards the partial fulfillment of the requirement for the degree of Doctor of Philosophy. Huntington's Disease (HD) belongs to the CAG repeat family of neurodegenerative diseases and is characterized by the presence of an expan...
متن کاملDynamic recruitment of active proteasomes into polyglutamine initiated inclusion bodies.
Neurodegenerative disorders such as Huntington's disease are hallmarked by neuronal intracellular inclusion body formation. Whether proteasomes are irreversibly recruited into inclusion bodies in these protein misfolding disorders is a controversial subject. In addition, it has been proposed that the proteasomes may become clogged by the aggregated protein fragments, leading to impairment of th...
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ژورنال
عنوان ژورنال: Movement Disorders
سال: 2017
ISSN: 0885-3185
DOI: 10.1002/mds.27226