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

Abstract:

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 over-expression of wild-type HspB8 in motor neuron-like NSC34 cells led to an incre...

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Authors


Phadwal, K More by this author
Turner, BJ More by this author
Oliver, PL More by this author
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Journal:
Journal of Neurochemistry
Volume:
119
Issue:
6
Pages:
1155-1161
Publication date:
2011-12-05
DOI:
EISSN:
1471-4159
ISSN:
0022-3042
URN:
uuid:841434cb-2020-4218-bb7a-149539f32cc9
Source identifiers:
221591
Local pid:
pubs:221591

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