Amyotrophic
lateral
sclerosis
(ALS)
is
a
fatal
neuromuscular
disorder
characterized
by
progressive
weakness
of
almost
all
skeletal
muscles,
whereas
extraocular
muscles
(EOMs)
are
comparatively
spared.
While
hindlimb
and
diaphragm
end-stage
SOD1G93A
(G93A)
mice
(a
familial
ALS
mouse
model)
exhibit
severe
denervation
depletion
Pax7+satellite
cells
(SCs),
we
found
that
the
pool
SCs
integrity
junctions
(NMJs)
maintained
in
EOMs.
In
cell
sorting
profiles,
derived
from
G93A
denervation-related
activation,
EOMs
display
spontaneous
(non-denervation-related)
similar
to
wild-type
mice.
Specifically,
cultured
EOM
contain
more
abundant
transcripts
axon
guidance
molecules,
including
Cxcl12,
along
with
sustainable
renewability
than
counterparts
under
differentiation
pressure.
co-culture
assays,
AAV-delivery
Cxcl12
G93A-hindlimb
SC-derived
myotubes
enhances
motor
neuron
extension
innervation,
recapitulating
innervation
capacity
myotubes.
fed
sodium
butyrate
(NaBu)
supplementation
exhibited
less
NMJ
loss
muscles.
Additionally,
displayed
elevated
expression
improved
following
NaBu
treatment
vitro.
Thus,
NaBu-induced
transcriptomic
changes
resembling
patterns
may
underlie
beneficial
effects
observed
More
broadly,
distinct
profile
offer
novel
therapeutic
targets
slow
functional
decay
provide
possible
"response
biomarkers"
pre-clinical
clinical
studies.
The Neuroscientist,
Год журнала:
2022,
Номер
30(1), С. 71 - 86
Опубликована: Сен. 17, 2022
Vascular
endothelial
growth
factor
(VEGF)
is
well
known
for
its
angiogenic
activity,
but
recent
evidence
has
revealed
a
neuroprotective
action
of
this
on
injured
or
diseased
neurons.
In
the
present
review,
we
summarize
most
relevant
findings
that
have
contributed
to
establish
link
between
VEGF
deficiency
and
neuronal
degeneration.
At
issue,
1)
mutant
mice
with
reduced
levels
show
adult-onset
muscle
weakness
motoneuron
degeneration
resembling
amyotrophic
lateral
sclerosis
(ALS),
2)
administration
different
animal
models
improves
motor
performance
ameliorates
motoneuronal
degeneration,
3)
there
an
association
low
plasmatic
human
ALS.
Altogether,
results
presented
in
review
highlight
as
essential
neurotrophic
endowed
promising
therapeutic
potential
treatment
disorders.
Amyotrophic
lateral
sclerosis
(ALS)
is
a
fatal
neuromuscular
disorder
characterized
by
progressive
weakness
of
almost
all
skeletal
muscles,
whereas
extraocular
muscles
(EOMs)
are
comparatively
spared.
While
hindlimb
and
diaphragm
end-stage
SOD1G93A
(G93A)
mice
(a
familial
ALS
mouse
model)
exhibit
severe
denervation
depletion
Pax7
+
satellite
cells
(SCs),
we
found
that
the
pool
SCs
integrity
junctions
(NMJs)
maintained
in
EOMs.
In
cell
sorting
profiles,
derived
from
G93A
denervation-related
activation,
EOMs
display
spontaneous
(non-denervation-related)
similar
to
wild-type
mice.
Specifically,
cultured
EOM
contain
more
abundant
transcripts
axon
guidance
molecules,
including
Cxcl12
,
along
with
sustainable
renewability
than
counterparts
under
differentiation
pressure.
co-culture
assays,
AAV-delivery
G93A-hindlimb
SC-derived
myotubes
enhances
motor
neuron
extension
innervation,
recapitulating
innervation
capacity
myotubes.
fed
sodium
butyrate
(NaBu)
supplementation
exhibited
less
NMJ
loss
muscles.
Additionally,
displayed
elevated
expression
improved
following
NaBu
treatment
vitro.
Thus,
NaBu-induced
transcriptomic
changes
resembling
patterns
may
contribute
beneficial
effects
observed
More
broadly,
distinct
profile
offer
novel
therapeutic
targets
slow
functional
decay
provide
possible
‘response
biomarkers’
pre-clinical
clinical
studies.
Applied Sciences,
Год журнала:
2022,
Номер
12(16), С. 7970 - 7970
Опубликована: Авг. 9, 2022
The
visual
organ
is
important
for
animals
to
obtain
information
and
understand
the
outside
world;
however,
robots
cannot
do
so
without
a
system.
At
present,
vision
technology
of
artificial
intelligence
has
achieved
automation
relatively
simple
intelligence;
bionic
equipment
not
as
dexterous
intelligent
human
eye.
can
function
smartly
beings;
existing
reviews
robot
are
still
limited.
Robot
been
explored
in
view
humans
animals’
principles
motion
characteristics.
In
this
study,
development
history
related
technologies
discussed,
most
representative
binocular
multi-eye
compound
eye
selected,
reviewed;
their
prospects
discussed
from
perspective
control.
This
comprehensive
study
will
serve
up-to-date
source
regarding
developments
field
technology.
Frontiers in Neuroanatomy,
Год журнала:
2024,
Номер
18
Опубликована: Июнь 18, 2024
Introduction
Extraocular
muscles
are
innervated
by
two
anatomically
and
histochemically
distinct
motoneuron
populations:
motoneurons
of
multiply-innervated
fibers
(MIF),
singly-innervated
(SIF).
Recently,
it
has
been
established
our
research
group
that
these
types
monkey
abducens
trochlear
nuclei
express
ion
channel
profiles:
SIF
motoneurons,
as
well
internuclear
neurons
(INT),
strong
Kv1.1
Kv3.1b
immunoreactivity,
indicating
their
fast-firing
capacity,
whereas
MIF
do
not.
Moreover,
low
voltage
activated
cation
channels,
such
Cav3.1
HCN1
showed
differences
between
post-inhibitory
rebound
characteristics.
However,
the
profiles
have
not
in
human
brainstem
tissue.
Methods
Therefore,
we
used
immunohistochemical
methods
with
antibodies
against
Kv,
Cav3
HCN
channels
to
(1)
examine
nucleus
terms
anatomical
organization
(2)
immunolabeling
patterns
proteins
populations
nuclei.
Results
In
examination
nucleus,
a
third
subgroup
was
consistently
encountered
weak
perineuronal
nets
(PN).
The
this
had
-on
average-
larger
diameters
than
smaller
PN
expression
strength
correlated
neuronal
size.
Immunolabeling
various
revealed
that,
general,
did
differ
consistently,
opposed
findings
Kv1.1,
were
found
on
both
density
varied
for
multiple
channels.
On
other
hand,
significant
INTs
immunoreactivity.
Discussion
These
results
indicated
may
be
more
variable
histochemical
biophysiological
characteristics,
previously
thought.
This
study
therefore
establishes
grounds
any
motor
controlling
extraocular
eye
movement
related
pathologies
brainstem.
Neural Regeneration Research,
Год журнала:
2022,
Номер
18(8), С. 0 - 0
Опубликована: Дек. 11, 2022
Vascular
endothelial
growth
factor
(VEGF)
was
discovered
by
its
angiogenic
activity.
However,
during
evolution,
it
appeared
earlier
as
a
neurotrophic
required
for
the
development
of
nervous
system
in
invertebrates
lacking
circulatory
system.
We
aimed
at
reviewing
recent
evidence
indicating
that
VEGF
has
neuroprotective
effects
neurons
exposed
to
variety
insults.
Of
particular
interest
is
link
established
between
and
motoneurons,
especially
after
design
VEGFδ/δ
mutant
mice.
These
mice
are
characterized
low
levels
develop
muscle
weakness
motoneuron
degeneration
resembling
amyotrophic
lateral
sclerosis.
The
administration
through
several
routes
animal
models
sclerosis
delays
motor
impairment
increases
life
expectancy.
There
new
advances
role
physiology
motoneurons.
Our
experimental
aims
use
extraocular
(abducens)
motoneurons
lesioned
axotomy
model
studying
actions.
Axotomized
abducens
exhibit
severe
alterations
their
discharge
activity
loss
synaptic
boutons.
exogenous
axotomized
either
from
transected
nerve
or
intraventricularly,
fully
restores
properties
despite
being
axotomized.
In
addition,
when
an
anti-VEGF
neutralizing
antibody
delivered
intact,
uninjured
these
cells
display
pattern
boutons
resemble
state
axotomy.
All
data
indicate
essential
Journal of Neurology,
Год журнала:
2023,
Номер
271(1), С. 325 - 339
Опубликована: Сен. 15, 2023
Abstract
Amyotrophic
lateral
sclerosis
(ALS)
can
result
into
an
incomplete
locked
in
state
(iLIS),
which
communication
depends
on
eye
tracking
computer
devices.
Oculomotor
function
impairments
ALS
have
been
reported,
but
there
is
little
research,
particularly
with
respect
to
patients
iLIS.
In
the
present
study,
we
compared
reflexive
and
executive
oculomotor
by
means
of
test
battery
between
three
groups:
advanced
iLIS
(n
=
22),
early
middle
stages
44)
healthy
subjects
32).
Patients
showed
significant
deteriorations
functions,
stronger
More
specifically,
produced
visually
guided
prosaccades
longer
latencies
more
frequent
hypometria
subjects.
Longest
were
obtained
patients,
a
prolongation
for
vertical
than
horizontal
prosaccades.
made
antisaccade
errors
generated
antisaccades
latencies.
Smooth
pursuit
was
also
impaired
ALS.
earlier
stages,
bulbar
onset
presented
smooth
deficits
spinal
patients.
Our
findings
reveal
relevant
deterioration
important
functions
ALS,
increases
It
includes
movements
loss
inhibitory
control,
indicating
progressing
pathological
involvement
prefrontal,
midbrain
brainstem
areas.
The
assessment
may
therefore
provide
clinically
bio-
progression
marker,
