Pharmaceuticals,
Journal Year:
2024,
Volume and Issue:
17(10), P. 1286 - 1286
Published: Sept. 27, 2024
Cu/Zn
Superoxide
Dismutase
1
(SOD1)
is
a
32
kDa
cytosolic
dimeric
metalloenzyme
that
neutralizes
superoxide
anions
into
oxygen
and
hydrogen
peroxide.
Mutations
in
SOD1
are
associated
with
ALS,
disease
causing
motor
neuron
atrophy
subsequent
mortality.
These
mutations
exert
their
harmful
effects
through
gain
of
function
mechanism,
rather
than
loss
function.
Despite
extensive
research,
the
mechanism
selective
death
still
remains
unclear.
A
defining
feature
ALS
pathogenesis
protein
misfolding
aggregation,
evidenced
by
ubiquitinated
inclusions
containing
affected
neurons.
This
work
aims
to
identify
compounds
countering
SOD1(A4V)
which
could
potentially
aid
treatment.
Cells,
Journal Year:
2025,
Volume and Issue:
14(4), P. 276 - 276
Published: Feb. 13, 2025
Mitochondrial
dysfunction
represents
a
pivotal
characteristic
of
numerous
neurodegenerative
disorders,
including
Alzheimer's
disease,
Parkinson's
Huntington's
and
amyotrophic
lateral
sclerosis.
These
conditions,
distinguished
by
unique
clinical
pathological
features,
exhibit
shared
pathways
leading
to
neuronal
damage,
all
which
are
closely
associated
with
mitochondrial
dysfunction.
The
high
metabolic
requirements
neurons
make
even
minor
deficiencies
highly
impactful,
driving
oxidative
stress,
energy
deficits,
aberrant
protein
processing.
Growing
evidence
from
genetic,
biochemical,
cellular
investigations
associates
impaired
electron
transport
chain
activity
disrupted
quality-control
mechanisms,
such
as
mitophagy,
the
initial
phases
disease
progression.
Furthermore,
overproduction
reactive
oxygen
species
persistent
neuroinflammation
can
establish
feedforward
cycles
that
exacerbate
deterioration.
Recent
research
has
increasingly
focused
on
interventions
aimed
at
enhancing
resilience-through
antioxidants,
small
molecules
modulate
balance
fusion
fission,
or
gene-based
therapeutic
strategies.
Concurrently,
initiatives
identify
dependable
biomarkers
seek
detect
changes
prior
manifestation
overt
symptoms.
By
integrating
current
body
knowledge,
this
review
emphasizes
critical
role
preserving
homeostasis
viable
approach.
It
also
addresses
complexities
translating
these
findings
into
practice
underscores
potential
innovative
strategies
designed
delay
potentially
halt
processes.
Antioxidants,
Journal Year:
2024,
Volume and Issue:
13(9), P. 1062 - 1062
Published: Aug. 30, 2024
Neurodegenerative
diseases
encompass
a
spectrum
of
disorders
marked
by
the
progressive
degeneration
structure
and
function
nervous
system.
These
conditions,
including
Parkinson's
disease
(PD),
Alzheimer's
(AD),
Huntington's
(HD),
Amyotrophic
lateral
sclerosis
(ALS),
Multiple
(MS),
often
lead
to
severe
cognitive
motor
deficits.
A
critical
component
neurodegenerative
pathologies
is
imbalance
between
pro-oxidant
antioxidant
mechanisms,
culminating
in
oxidative
stress.
The
brain's
high
oxygen
consumption
lipid-rich
environment
make
it
particularly
vulnerable
damage.
Pro-oxidants
such
as
reactive
nitrogen
species
(RNS)
(ROS)
are
continuously
generated
during
normal
metabolism,
counteracted
enzymatic
non-enzymatic
defenses.
In
diseases,
this
balance
disrupted,
leading
neuronal
This
systematic
review
explores
roles
stress,
gut
microbiota,
epigenetic
modifications
aiming
elucidate
interplay
these
factors
identify
potential
therapeutic
strategies.
We
conducted
comprehensive
search
articles
published
2024
across
major
databases,
focusing
on
studies
examining
relationships
redox
homeostasis,
changes
neurodegeneration.
total
161
were
included,
comprising
clinical
trials,
observational
studies,
experimental
research.
Our
findings
reveal
that
stress
plays
central
role
pathogenesis
with
microbiota
composition
significantly
influencing
balance.
Specific
bacterial
taxa
markers
identified
modulators
suggesting
novel
avenues
for
intervention.
Moreover,
recent
evidence
from
human
animal
supports
emerging
concept
targeting
homeostasis
through
therapies.
Future
research
should
focus
validating
targets
settings
exploring
personalized
medicine
strategies
based
individual
profiles.
Signal Transduction and Targeted Therapy,
Journal Year:
2025,
Volume and Issue:
10(1)
Published: Jan. 6, 2025
Cells
orchestrate
their
processes
through
complex
interactions,
precisely
organizing
biomolecules
in
space
and
time.
Recent
discoveries
have
highlighted
the
crucial
role
of
biomolecular
condensates-membrane-less
assemblies
formed
condensation
proteins,
nucleic
acids,
other
molecules-in
driving
efficient
dynamic
cellular
processes.
These
condensates
are
integral
to
various
physiological
functions,
such
as
gene
expression
intracellular
signal
transduction,
enabling
rapid
finely
tuned
responses.
Their
ability
regulate
signaling
pathways
is
particularly
significant,
it
requires
a
careful
balance
between
flexibility
precision.
Disruption
this
can
lead
pathological
conditions,
including
neurodegenerative
diseases,
cancer,
viral
infections.
Consequently,
emerged
promising
therapeutic
targets,
with
potential
offer
novel
approaches
disease
treatment.
In
review,
we
present
recent
insights
into
regulatory
mechanisms
by
which
influence
pathways,
roles
health
disease,
strategies
for
modulating
condensate
dynamics
approach.
Understanding
these
emerging
principles
may
provide
valuable
directions
developing
effective
treatments
targeting
aberrant
behavior
diseases.
Preparative Biochemistry & Biotechnology,
Journal Year:
2025,
Volume and Issue:
unknown, P. 1 - 21
Published: March 27, 2025
Cu/Zn
Superoxide
Dismutase
(SOD1)
plays
a
critical
role
in
alleviating
oxidative
stress
by
catalyzing
the
conversion
of
superoxide
radicals
into
oxygen
and
hydrogen
peroxide.
This
review
presents
an
in-depth
analysis
challenges
strategies
involved
optimizing
SOD1
production,
with
focus
on
Pichia
pastoris
as
expression
system.
Key
approaches
such
strategic
selection
vectors,
codon
optimization,
fine-tuning
fermentation
parameters
to
maximize
yield
are
thoroughly
explored.
Advances
protein
engineering,
co-expression
molecular
chaperones,
use
stabilizers
additives
also
examined
for
their
improving
stability
functionality.
The
highlights
significant
biomedical
industrial
applications
overexpressed
thermostable
SOD1,
uncovering
novel
opportunities
therapeutic
interventions
biotechnological
innovations.
Additionally,
emerging
technologies
omics-based
approaches,
advanced
engineering
tools,
alternative
host
systems
discussed,
offering
new
avenues
future
research.
comprehensive
underscores
transformative
potential
positioning
it
at
forefront
scientific
technological
advancements.
Genes,
Journal Year:
2025,
Volume and Issue:
16(4), P. 407 - 407
Published: March 30, 2025
Amyotrophic
lateral
sclerosis
(ALS)
is
a
devastating
neurodegenerative
disorder
characterized
by
the
progressive
degeneration
of
motor
neurons,
leading
to
muscle
weakness,
paralysis,
and
death.
Although
significant
progress
has
been
made
in
understanding
ALS,
its
molecular
mechanisms
remain
complex
multifactorial.
This
review
explores
potential
convergent
underlying
ALS
pathogenesis,
focusing
on
roles
key
proteins
including
NEK1,
C21ORF2,
cyclin
F,
VCP,
TDP-43.
Recent
studies
suggest
that
mutations
C21ORF2
lead
stabilization
while
F
activate
resulting
TDP-43
aggregation.
aggregation,
hallmark
impairs
RNA
processing
protein
transport,
both
which
are
essential
for
neuronal
function.
Furthermore,
emerged
as
player
DNA
damage
repair,
translocating
sites
recruiting
repair
proteins.
Given
also
involved
this
examines
how
these
may
intersect
disrupt
mechanisms,
contributing
progression.
Impaired
homeostasis
suggested
be
central
downstream
pathogenesis.
Ultimately,
interplay
between
pathways
could
offer
novel
insights
into
provide
therapeutic
targets.
aims
highlight
emerging
connections
cellular
dysfunction
fostering
deeper
basis
avenues
intervention.
