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@ARTICLE{Zhu:137195,
author = {Zhu, Jun-Yi and Vereshchagina, Natalia and Sreekumar,
Vrinda and Burbulla, Lena F and Costa, Ana C and Daub,
Katharina J and Woitalla, Dirk and Martins, L Miguel and
Krüger, Rejko and Rasse, Tobias M},
title = {{K}nockdown of {H}sc70-5/mortalin induces loss of synaptic
mitochondria in a {D}rosophila {P}arkinson's disease model.},
journal = {PLOS ONE},
volume = {8},
number = {12},
issn = {1932-6203},
address = {San Francisco, California, US},
publisher = {PLOS},
reportid = {DZNE-2020-03517},
pages = {e83714},
year = {2013},
abstract = {Mortalin is an essential component of the molecular
machinery that imports nuclear-encoded proteins into
mitochondria, assists in their folding, and protects against
damage upon accumulation of dysfunctional, unfolded proteins
in aging mitochondria. Mortalin dysfunction associated with
Parkinson's disease (PD) increases the vulnerability of
cultured cells to proteolytic stress and leads to changes in
mitochondrial function and morphology. To date, Drosophila
melanogaster has been successfully used to investigate
pathogenesis following the loss of several other
PD-associated genes. We generated the first
loss-of-Hsc70-5/mortalin-function Drosophila model. The
reduction of Mortalin expression recapitulates some of the
defects observed in the existing Drosophila PD-models, which
include reduced ATP levels, abnormal wing posture, shortened
life span, and reduced spontaneous locomotor and climbing
ability. Dopaminergic neurons seem to be more sensitive to
the loss of mortalin than other neuronal sub-types and
non-neuronal tissues. The loss of synaptic mitochondria is
an early pathological change that might cause later
degenerative events. It precedes both behavioral
abnormalities and structural changes at the neuromuscular
junction (NMJ) of mortalin-knockdown larvae that exhibit
increased mitochondrial fragmentation. Autophagy is
concomitantly up-regulated, suggesting that mitochondria are
degraded via mitophagy. Ex vivo data from human fibroblasts
identifies increased mitophagy as an early pathological
change that precedes apoptosis. Given the specificity of the
observed defects, we are confident that the loss-of-mortalin
model presented in this study will be useful for further
dissection of the complex network of pathways that underlie
the development of mitochondrial parkinsonism.},
keywords = {Animals / Autophagy: genetics / Cell Survival: genetics /
Disease Models, Animal / Dopaminergic Neurons: metabolism /
Drosophila / Female / Gene Knockdown Techniques / Gene
Silencing / Genes, Essential / HSP70 Heat-Shock Proteins:
genetics / Humans / Mitochondria: genetics / Mitochondria:
metabolism / Neurons: metabolism / Parkinson Disease:
genetics / Parkinson Disease: metabolism / Phenotype /
Synapses: metabolism / HSP70 Heat-Shock Proteins (NLM
Chemicals) / mortalin (NLM Chemicals)},
cin = {AG Gasser 1},
ddc = {610},
cid = {I:(DE-2719)1210000},
pnm = {345 - Population Studies and Genetics (POF3-345)},
pid = {G:(DE-HGF)POF3-345},
typ = {PUB:(DE-HGF)16},
pubmed = {pmid:24386261},
pmc = {pmc:PMC3875477},
doi = {10.1371/journal.pone.0083714},
url = {https://pub.dzne.de/record/137195},
}