G392E neuroserpin causing the dementia FENIB is secreted from cells but is not synaptotoxic
نویسندگان
چکیده
منابع مشابه
CD93 is Selectively Expressed on Human Myeloma Cells but Not on B Lymphocytes
Background: CD93 has originally been known as a C1q receptor, and many studies have demonstrated that CD93 is expressed on hematopoietic stem cells, B cell progenitors, myeloid and monocytic cells. Moreover, CD93 is shown to be expressed on long-lived plasma cells, and CD93 deficient-mice display an impairment in plasma cell development. Objective: To investiga...
متن کاملMutation-, aging-, and gene dosage-dependent accumulation of neuroserpin (G392E) in endoplasmic reticula and lysosomes of neurons in transgenic mice.
Mutations in human neuroserpin gene cause an autosomal dementia, familial encephalopathy with neuroserpin inclusion bodies (FENIB). We generated and analyzed transgenic mice expressing high levels of either FENIB-type (G392E) or wild-type human neuroserpin in neurons of the central nervous system. G392E neuroserpin accumulated age-dependently in neurons of the neocortex, thalamus, amygdala, pon...
متن کاملNeuroserpin 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...
متن کاملPolymer toxicity in neurodegeneration FENIB
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...
متن کاملThe intracellular accumulation of polymeric neuroserpin explains the severity of the dementia FENIB
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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ژورنال
عنوان ژورنال: Scientific Reports
سال: 2021
ISSN: 2045-2322
DOI: 10.1038/s41598-021-88090-1