Neuroserpin polymers cause oxidative stress in a neuronal model of the dementia FENIB
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منابع مشابه
Neuroserpin polymers cause oxidative stress in a neuronal model of the dementia FENIB
The serpinopathies are human pathologies caused by mutations that promote polymerisation and intracellular deposition of proteins of the serpin superfamily, leading to a poorly understood cell toxicity. The dementia FENIB is caused by polymerisation of the neuronal serpin neuroserpin (NS) within the endoplasmic reticulum (ER) of neurons. With the aim of understanding the toxicity due to intrace...
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Many neurodegenerative conditions, including Alzheimer’s, Parkinson’s and Huntington’s diseases, the prion encephalopathies and amyotrophic lateral sclerosis, are now recognised as protein conformational diseases, an ample group of pathologies characterised by the transition of wild type or mutated proteins to aggregationprone conformations. This leads to their intracellular and/ or extracellul...
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Familial encephalopathy with neuroserpin inclusion bodies (FENIB) is an autosomal dominant dementia that is characterized by the retention of polymers of neuroserpin as inclusions within the endoplasmic reticulum (ER) of neurons. We have developed monoclonal antibodies that detect polymerized neuroserpin and have used COS-7 cells, stably transfected PC12 cell lines and transgenic Drosophila mel...
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The serpinopathies result from conformational transitions in members of the serine proteinase inhibitor superfamily with aberrant tissue deposition or loss of function. They are typified by mutants of neuroserpin that are retained within the endoplasmic reticulum of neurons as ordered polymers in association with dementia. We show here that the S49P mutant of neuroserpin that causes the dementi...
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Mutants of neuroserpin are retained as polymers within the endoplasmic reticulum (ER) of neurones to cause the autosomal dominant dementia familial encephalopathy with neuroserpin inclusion bodies or FENIB. The cellular consequences are unusual in that the ordered polymers activate the ER overload response (EOR) in the absence of the canonical unfolded protein response. We use both cell lines a...
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ژورنال
عنوان ژورنال: Neurobiology of Disease
سال: 2017
ISSN: 0969-9961
DOI: 10.1016/j.nbd.2017.03.010