Publication: Neurons other than motor neurons in motor neuron disease
Authors
Ruffoli, Riccardo ; Biagioni, Francesca ; Busceti, Carla L. ; Gaglione, Anderson ; Ryskalin, Larisa ; Gambardella, Stefano ; Frati, Alessandro ; Fornai, Francesco
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Publisher
Universidad de Murcia. Departamento de BiologĂa Celular e HistologĂa
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DOI
DOI: 10.14670/HH-11-895
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info:eu-repo/semantics/article
Description
Abstract
Amyotrophic lateral sclerosis (ALS) is
typically defined by a loss of motor neurons in the
central nervous system. Accordingly, morphological
analysis for decades considered motor neurons (in the
cortex, brainstem and spinal cord) as the neuronal
population selectively involved in ALS. Similarly, this
was considered the pathological marker to score disease
severity ex vivo both in patients and experimental
models. However, the concept of non-autonomous motor
neuron death was used recently to indicate the need for
additional cell types to produce motor neuron death in
ALS. This means that motor neuron loss occurs only
when they are connected with other cell types. This
concept originally emphasized the need for resident glia
as well as non-resident inflammatory cells. Nowadays,
the additional role of neurons other than motor neurons
emerged in the scenario to induce non-autonomous
motor neuron death. In fact, in ALS neurons diverse
from motor neurons are involved. These cells play
multiple roles in ALS: (i) they participate in the chain of
events to produce motor neuron loss; (ii) they may even
degenerate more than and before motor neurons.
In the present manuscript evidence about multineuronal involvement in ALS patients and experimental
models is discussed. Specific sub-classes of neurons in
the whole spinal cord are reported either to degenerate or
to trigger neuronal degeneration, thus portraying ALS as
a whole spinal cord disorder rather than a disease
affecting motor neurons solely. This is associated with a
novel concept in motor neuron disease which recruits
abnormal mechanisms of cell to cell communication.
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Citation
Histology and Histopathology, Vol.32, nÂş11, (2017)
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