Intra-axonal protein aggregation in the peripheral nervous system.
J Peripher Nerv Syst. 2014-03-01; 19(1): 44-49
DOI: 10.1111/jns5.12056
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1. J Peripher Nerv Syst. 2014 Mar;19(1):44-9. doi: 10.1111/jns5.12056.
Intra-axonal protein aggregation in the peripheral nervous system.
Vital A(1), Meissner WG, Canron MH, Martin-Negrier ML, Bezard E, Tison F, Vital
C.
Author information:
(1)Université Bordeaux; CNRS, Institut des Maladies Neurodégénératives, Bordeaux,
France; Department of Pathology, Bordeaux University Hospital, Bordeaux, France.
Intracellular protein aggregates are common pathological hallmarks of many
neurodegenerative disorders, and a defect in axonal transport is also
incriminated. Here, we studied intra-axonal abnormal protein aggregation and
axonopathy by using immunohistochemistry and electron microscopy on peripheral
nerve biopsies from 12 patients with chronic axonal peripheral neuropathy (PN) of
unknown etiology. Among these patients, three had idiopathic Parkinson’s disease
(PD). Intra-axonal ubiquitin aggregates were more numerous in the patients with
PD. Intra-axonal aggregates of tau AT8 were found in five patients without PD.
Phosphorylated α-synuclein aggregation was absent in all cases, while
intra-axonal colocalization of 14-3-3 β and ubiquitin was observed in two PD
cases. Electron microscopy revealed enlarged axons crowded with organelles in six
cases, including the three patients with PD, thus attesting a slowing of the
axoplasmic flux. The number of ubiquitin aggregates was correlated with features
of reduced axonal flux, while no such correlation was found for tau and 14-3-3 β.
Age did not correlate with the number of tau, ubiquitin, and 14-3-3 aggregates.
Thus, both ubiquitin and/or abnormal tau intra-axonal aggregates may be found in
chronic axonal PN. Ubiquitin aggregates might reduce the axonal flux or result
from a disease producing slowing of axonal transport.
© 2014 Peripheral Nerve Society.
DOI: 10.1111/jns5.12056
PMID: 24494664 [Indexed for MEDLINE]