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Published byMarco Antônio Barata Marroquim Modified over 6 years ago
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Huntingtin Fibrils Poke Membranes
Pedro Guedes-Dias, Erika L.F. Holzbaur Cell Volume 171, Issue 1, Pages (September 2017) DOI: /j.cell Copyright © 2017 Elsevier Inc. Terms and Conditions
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Figure 1 Mutant Huntingtin (Htt) Forms Fibrillary Inclusion Bodies (IBs) In Situ Bäuerlein et al. use cryo-electron tomography (cryo-ET) to reveal the ultrastructure of mutant Htt IBs in primary neurons and HeLa cells expressing an N-terminal fragment of Htt encoding exon 1 that includes a pathological polyQ stretch of 97Q. The authors identify a similar structure of radially arranged fibrils in nuclear and cytosolic IBs and show that cytosolic IBs significantly interact and disrupt endoplasmic reticulum (ER) membranes. Their work provides evidence for a pathogenic role of mutant Htt IBs in Huntington’s disease. Cell , 32-33DOI: ( /j.cell ) Copyright © 2017 Elsevier Inc. Terms and Conditions
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