Huntingtin Fibrils Poke Membranes

Cell. 2017 Sep 21;171(1):32-33. doi: 10.1016/j.cell.2017.09.009.

Abstract

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.

Publication types

  • Comment

MeSH terms

  • Animals
  • Endoplasmic Reticulum
  • Humans
  • Huntingtin Protein*
  • Huntington Disease
  • Nerve Tissue Proteins / chemistry*
  • Nuclear Proteins / chemistry

Substances

  • Huntingtin Protein
  • Nerve Tissue Proteins
  • Nuclear Proteins