Research Square (Research Square),
Journal Year:
2023,
Volume and Issue:
unknown
Published: Sept. 20, 2023
Abstract
Background:
Amyotrophic
lateral
sclerosis
(ALS)
is
a
neurodegenerative
disease,
as
the
limitations
of
drug
therapies,
there
an
increasing
focus
on
rehabilitation
approach
that
includes
Physical
training
(PT)
and
Tuina
therapy.
This
study
aims
to
investigate
protective
effects
PT
therapy
physical
function,
Lung
quality
life
in
patients
with
ALS.
Methods:
randomized
controlled
trial.
Patients
will
be
divided
into
experimental
group
or
control
at
1:1
ratio,
each
both
have
oral
Riluzole.
The
conduct
for
4
weeks,
while
instructed
perform
basic
stretching
exercises
upper
lower
limbs
home
weeks.
ALS
Functional
Rating
Scale-Revised
scores
(ALSFRS-R),
Manual
muscle
test
(MMT),
Modified
Ashworth
spasm
scale
(MAS),
Forced
vital
capacity
(FVC),
Vital
(VC),
expiratory
volume
one
second
(FEV1),
FEV1/FVC,
Peak
flow
(PEF),
Maximal
voluntary
ventilation
(MVV),
Assessment
Questionnaire
(ALSAQ-40)
scores)
safety
measures
collected
baseline,
after
during
follow-ups
every
6
months
2
years.
Discussion:
provide
evidence-based
medical
corroboration
clinical
application
incorporating
promote
development
this
Comprehensive
early
rehabilitation.
Trial
registration:
Chinese
trial
registry,
ChiCTR2300068650.
Date:
2023-02-27.
https://www.chictr.org.cn/showproj.html?proj=186359
Ageing Research Reviews,
Journal Year:
2024,
Volume and Issue:
98, P. 102320 - 102320
Published: May 6, 2024
Aging
is
a
gradual
and
irreversible
natural
process.
With
aging,
the
body
experiences
functional
decline,
effects
amplify
vulnerability
to
range
of
age-related
diseases,
including
neurodegenerative,
cardiovascular,
metabolic
diseases.
Within
aging
process,
morphology
function
mitochondria
endoplasmic
reticulum
(ER)
undergo
alterations,
particularly
in
structure
connecting
these
organelles
known
as
mitochondria-associated
membranes
(MAMs).
MAMs
serve
vital
intracellular
signaling
hubs,
facilitating
communication
between
ER
when
regulating
various
cellular
events,
calcium
homeostasis,
lipid
metabolism,
mitochondrial
function,
apoptosis.
The
formation
partly
dependent
on
interaction
vesicle-associated
membrane
protein-associated
protein-B
(VAPB)
protein
tyrosine
phosphatase-interacting
protein-51
(PTPIP51).
Accumulating
evidence
has
begun
elucidate
pivotal
role
VAPB-PTPIP51
tether
initiation
progression
In
this
study,
we
delineate
intricate
multifunctional
discuss
its
profound
implications
aging-associated
Moreover,
provide
comprehensive
overview
potential
therapeutic
interventions
pharmacological
agents
targeting
VAPB-PTPIP51-mediated
MAMs,
thereby
offering
glimmer
hope
mitigating
processes
treating
disorders.
Annals of Clinical and Experimental Neurology,
Journal Year:
2025,
Volume and Issue:
19(1), P. 53 - 61
Published: April 3, 2025
Amyotrophic
lateral
sclerosis
(ALS)
is
a
progressive
neurodegenerative
disorder
characterized
by
the
death
of
upper
and
lower
motor
neurons.
Numerous
studies
show
that
structural
functional
impairments
neuromuscular
junctions
(NMJ)
occur
as
early
presymptomatic
stage
ALS.
NMJ
involvement
independent
one
primary
events
in
ALS
pathogenesis.
Aim:
to
review
data
on
characteristics
mechanisms
dysfunction
at
pre-
postsynaptic
levels
patients
transgenic
animal
model
disease.
Furthermore,
we
report
perisynaptic
Schwann
cells
impaired
neuron
skeletal
muscle
interaction
ALS,
with
focus
reviewed
publications
targeting
molecular
underlying
disruption
The
may
be
potential
target
for
novel
therapeutic
approaches
International Journal of Molecular Sciences,
Journal Year:
2024,
Volume and Issue:
25(16), P. 8959 - 8959
Published: Aug. 17, 2024
Age-related
impairment
of
the
diaphragm
causes
respiratory
complications.
Neuromuscular
junction
(NMJ)
dysfunction
can
be
one
triggering
events
in
weaknesses
old
age.
Prominent
structural
and
functional
alterations
NMJs
were
described
elderly
rodents,
but
NMJ
changes
middle
age
remain
unclear.
Here,
we
compared
muscles
from
young
adult
(3
months)
middle-aged
(12
BALB/c
mice.
Microelectrode
recordings,
immunofluorescent
staining,
electron
microscopy,
myography,
whole-body
plethysmography
used.
We
revealed
presynaptic
(i)
postsynaptic
(ii)
changes.
The
former
included
an
increase
both
action
potential
propagation
velocity
neurotransmitter
release
evoked
by
low-,
moderate-,
high-frequency
activity
a
decrease
immunoexpression
synapsin
1
synaptic
vesicle
clustering.
latter
consisted
currents
via
nicotinic
acetylcholine
receptors
area
their
distribution.
These
correlated
with
increased
contractile
responses
to
moderate-
nerve
activation.
Additionally,
found
pattern
respiration
(an
peak
inspiratory
flow
tendency
elevation
tidal
volume),
which
imply
conclude
that
enhancement
neuromuscular
communication
(due
mechanism)
accompanied
improved
occurs
early
aging.
Frontiers in Cell and Developmental Biology,
Journal Year:
2024,
Volume and Issue:
12
Published: June 20, 2024
Familial
amyotrophic
lateral
sclerosis
(ALS)
is
a
progressive
neuromuscular
disorder
that
due
to
mutations
in
one
of
several
target
genes,
including
SOD1
.
So
far,
clinical
records,
rodent
studies,
and
vitro
models
have
yielded
arguments
for
either
primary
motor
neuron
disease,
or
pleiotropic
pathogenesis
ALS.
While
mouse
lack
the
human
origin,
using
induced
pluripotent
stem
cells
(hiPSC)
been
recently
developed
addressing
ALS
pathogenesis.
In
spite
improvements
regarding
generation
muscle
from
hiPSC,
degree
maturation
resulting
these
protocols
has
remained
limited.
To
fill
shortcomings,
we
here
present
new
protocol
an
enhanced
myotube
differentiation
hiPSC
with
option
further
upon
coculture
hiPSC-derived
neurons.
The
described
model
first
yield
combination
key
myogenic
features
are
consistent
sarcomeric
organization
association
complex
nAChR
clusters
myotubes
derived
control
hiPSC.
this
model,
carrying
D90A
mutation
had
reduced
expression
markers,
sarcomeres,
morphologically
different
clusters,
altered
nAChR-dependent
Ca
2+
response
compared
myotubes.
Notably,
trophic
support
provided
by
neurons
cluster
differences
between
summary,
novel
yields
evidence
both
muscle-intrinsic
nerve-dependent
aspects
dysfunction
-based
Background:
TDP-43
aggregation
is
the
hallmark
pathology
seen
at
post-mortem
in
majority
of
Amyotrophic
lateral
sclerosis
(ALS)
cases.
Lipid
metabolism
has
been
shown
to
be
disrupted
ALS
including
evidence
for
systemic
metabolic
disruptions
patients
as
well
lipid
peroxidation
changes
noted
single
cell
level.
The
temporal
nature
dysregulation
during
disease
course
and
role
that
may
play
potentiating
unclear.
Aim:
aim
this
review
analyse
existing
literature
assessing
interplay
between
altered
ALS,
investigate
possible
relationships
two.
Methods:
This
protocol
describes
strategy
a
proposed
systematic
meta-analysis
identify,
evaluate
studies
examining
relation
pathology.
Summary:
will
aid
our
understanding
ALS-associated
pathobiology
through
detailed
analysis
molecular
processes
underlying
influence
lipids
on
behaviour
ALS.