Article
Amplification of neurotoxic HTTex1 assemblies in human neurons.
Neurobiology of disease - 1 Nov 2021
Chongtham Anjalika, Isas J Mario, Pandey Nitin K, Rawat Anoop, Yoo Jung Hyun, Mastro Tara, Kennedy Mary B, Langen Ralf, Khoshnan Ali
Abstract excerpt
Huntington's disease (HD) is a genetically inherited neurodegenerative disorder caused by expansion of a polyglutamine (polyQ) repeat in the exon-1 of huntingtin protein (HTT). The expanded polyQ enhances the amyloidogenic propensity of HTT exon 1 (HTTex1), which forms a heterogeneous mixture of assemblies with a broad neurotoxicity spectrum. While predominantly intracellular, monomeric and aggregated mutant HTT...
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