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Article

HspB8 mutation causing hereditary distal motor neuropathy impairs lysosomal delivery of autophagosomes.

Journal of neurochemistry - 1 Dec 2011

Kwok Alice S, Phadwal Kanchan, Turner Bradley J, Oliver Peter L, Raw Annie, Simon Anna Katharina, Talbot Kevin, Agashe Vishwas R

Abstract excerpt

HspB8, a small heat-shock protein implicated in autophagy, is mutated in patients with distal hereditary motor neuropathy type II (dHMNII). Autophagy is essential for maintaining protein homeostasis in the central nervous system, but its role has not been investigated in peripheral motor neurons. We used a novel, multispectral-imaging flow cytometry assay to measure autophagy in cells. This assay revealed that...

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Identifiers

PMID: 21985219
DOI: 10.1111/j.1471-4159.2011.07521.x

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