Nat Neurosci: Co-morbidity mechanisms associated with C9ORF72 in patients with defrostanda and prefrontal lobe dementia
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Last Update: 2020-05-30
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Source: Internet
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Author: User
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The coding prediction of hexanucleotide amplification of the otoid exchange factor C9ORF72 is the most common genetic cause of amyotrophic lateral sclerosis (ALS) and pre-temporal lobe dementia (FTD)although repeated amplification has been determined to produce toxic products, mRNAs encoded with the C9ORF72 protein will also decrease in affected individualsin the study, researchers tested how C9ORF72 protein levels affect the toxicity of repeated mediaagesIn 66 GGGGCC repeats of C9orf72 in vitro genetically modified mice, one or two endogenous C9ORF72 alleles inactivated induced or accelerated early deathinactivated one or two endogenous C9orf72 alleles in mice expressing 450 repetitive C9orf72 transgenies, exacerbated cognitive impairment, hippocampus neuron allotment, glial activation, and dipeptide regenerative protein accumulationreduced C9ORF72 has been shown to inhibit the rise of repetitive lying autophagythese results support a disease mechanism in ALS/FTD, where C9ORF72 is reduced and can lead to autophagy defects, in synergy with toxic gains from repetitive dependence
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