Open AccessHuntingtin Fibrils Poke Membranes
Author(s) -
Pedro Guedes-Dias,
Erika L.F. Holzbaur
Publication year - 2017
Publication title -
cell
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 26.304
H-Index - 776
eISSN - 1097-4172
pISSN - 0092-8674
DOI - 10.1016/j.cell.2017.09.009
Subject(s) - biology , huntingtin , microbiology and biotechnology , fibril , membrane , biophysics , genetics , gene , mutant
A hallmark of Huntington's disease is the presence of intracellular aggregates of mutant huntingtin, the pathological significance of which has long been debated. Using cryo-electron tomography, Bauerlein et al. reveal the fibrillary structure of huntingtin aggregates in situ and show that huntingtin fibrils interact with the endoplasmic reticulum, distorting its morphology and dynamics.
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