Cell Biochemistry and Function,
Год журнала:
2024,
Номер
42(8)
Опубликована: Дек. 1, 2024
ABSTRACT
Autophagy
is
an
essential
intracellular
degradation
system
responsible
for
delivering
cytoplasmic
components
to
lysosomes.
Within
this
intricate
process,
optineurin
(OPTN),
autophagy
receptor,
has
attracted
extensive
attention
due
its
multifaceted
roles
in
the
process.
OPTN
regulated
by
various
posttranslational
modifications
and
actively
participates
numerous
signaling
pathways
cellular
processes.
By
exploring
regulatory
mechanism
of
modification,
we
can
further
understand
critical
role
protein
modification
biological
progress,
such
as
autophagy.
Additionally,
implicated
many
human
diseases,
including
rheumatoid
arthritis,
osteoporosis,
infectious
diseases.
And
delve
into
inflammatory
clarify
how
it
regulates
diseases
cancer.
We
aim
enhance
understanding
OPTN's
functions
processes
implications
pathogenesis
DNA repair,
Год журнала:
2024,
Номер
138, С. 103679 - 103679
Опубликована: Апрель 16, 2024
Neurodegenerative
diseases
are
the
second
most
prevalent
cause
of
death
in
industrialized
countries.
Alzheimer's
Disease
is
widespread
and
also
acknowledged
form
dementia
today.
Together
with
Parkinson's
they
account
for
over
90
%
cases
neurodegenerative
disorders
caused
by
proteopathies.
Far
less
known
pathologies
DNA
repair
deficiency
syndromes.
Such
like
Cockayne
-
or
Werner
Syndrome
described
as
progeroid
syndromes
that
premature
ageing
affected
persons,
there
clear
implications
such
neurologic
dysfunction
degeneration.
In
this
review,
we
aim
to
draw
attention
on
commonalities
between
proteopathy-associated
neurodegeneration
defects
discuss
how
mitochondria
implicated
development
both
disorder
classes.
Furthermore,
highlight
nematodes
a
valuable
indispensable
model
organism
study
conserved
processes
fast-forward
manner.
International Journal of Molecular Sciences,
Год журнала:
2023,
Номер
24(13), С. 10776 - 10776
Опубликована: Июнь 28, 2023
The
retinal
pigment
epithelium
(RPE)
performs
a
range
of
necessary
functions
within
the
neural
layers
retina
and
helps
ensure
vision.
regulation
pro-oxidative
antioxidant
processes
is
basis
for
maintaining
RPE
homeostasis
preventing
degenerative
processes.
Long-term
stable
changes
in
redox
balance
under
influence
endogenous
or
exogenous
factors
can
lead
to
oxidative
stress
(OS)
development
number
pathologies
associated
with
dysfunction,
eventually
vision
loss.
Reparative
autophagy,
ubiquitin–proteasome
utilization,
repair
damaged
proteins,
maintenance
their
conformational
structure
are
important
interrelated
mechanisms
defense
system
that
protects
against
damage.
Antioxidant
protection
cells
realized
as
result
activity
specific
transcription
factors,
large
group
enzymes,
chaperone
etc.,
which
form
many
signaling
pathways
retina.
Here,
we
discuss
role
key
components
(ADS)
cellular
response
OS.
Understanding
interactions
OS
mediators
ADS
contributes
formation
ideas
about
subtle
prospects
experimental
approaches
restore
functions.
Frontiers in Molecular Neuroscience,
Год журнала:
2025,
Номер
18
Опубликована: Фев. 20, 2025
Mitochondrial
integrity
is
fundamental
to
cellular
function,
upheld
by
a
network
of
proteases
that
regulate
proteostasis
and
mitochondrial
dynamics.
Among
these
proteases,
AFG3L2
critical
due
its
roles
in
maintaining
homeostasis,
regulating
protein
quality,
facilitating
biogenesis.
Mutations
are
implicated
spectrum
diseases,
including
spinocerebellar
ataxia
type
28
(SCA28)
spastic
5
(SPAX5),
as
well
other
systemic
conditions.
This
study
employs
multi-omics
approach
investigate
the
biochemical
impact
mutations
immortalized
lymphoblastoid
cell
lines
derived
from
patient
with
biallelic
variants
leading
(SPAX5).
Our
proteomic
analysis
revealed
impairment,
significant
dysregulation
proteins
for
cytoskeletal
integrity,
metabolism.
Specifically,
disruptions
were
observed
dynamics
calcium
alongside
downregulation
key
like
COX11,
copper
chaperone
complex
IV
assembly,
NFU1,
an
iron-sulfur
cluster
linked
paraparesis
infection-related
worsening.
Lipidomic
highlighted
substantial
alterations
lipid
composition,
decreases
sphingomyelins,
phosphatidylethanolamine,
phosphatidylcholine,
reflecting
metabolism
membrane
integrity.
Metabolomic
profiling
did
not
reveal
any
findings.
comprehensive
investigation
into
loss
functional
elucidates
pathophysiology
extending
beyond
proteostasis,
implicating
wide
array
processes.
The
findings
disturbances
at
multiple
levels,
contributing
neurodegeneration
through
disrupted
respiratory
chain,
altered
homeostasis.
underscores
complexity
SPAX5
importance
approaches
developing
effective
strategies
address
AFG3L2.
data
also
highlight
value
cells
tool
pre-clinical
testing
research,
offering
detailed
fingerprint
enhances
our
understanding
identifies
potential
areas
further
investigation.
Chemosphere,
Год журнала:
2025,
Номер
377, С. 144348 - 144348
Опубликована: Апрель 9, 2025
Parkinson's
disease
(PD)
is
the
fastest-growing
neurodegenerative
disorder
worldwide,
and
no
effective
cure
currently
available.
Neuropathologically,
PD
characterized
by
selective
degeneration
of
dopaminergic
neurons
in
substantia
nigra
accumulation
alpha-synuclein
(aSyn)-rich
proteinaceous
inclusions
within
surviving
neurons.
As
a
multifactorial
disorder,
approximately
85
%
cases
are
sporadic
with
unknown
etiology.
Among
many
risk
factors
implicated
PD,
exposure
to
neurotoxic
pesticides
stands
out
as
significant
contributor.
While
effects
still
uncharacterized,
it
has
already
been
shown
that
rotenone,
paraquat,
maneb,
dieldrin
affect
critical
cellular
pathways,
including
mitochondrial
proteasomal
dysfunction,
aSyn
aggregation,
autophagy
dysregulation,
disruption
dopamine
metabolism.
With
constant
rise
pesticide
usage
meet
demands
growing
human
population,
environmental
contamination
subsequent
development
also
increasing.
This
review
explores
molecular
mechanisms
which
influences
development,
shedding
light
on
their
role
pathogenesis
highlighting
need
for
preventative
measures
regulatory
oversight
mitigate
these
risks.
Cardiovascular Innovations and Applications,
Год журнала:
2025,
Номер
10(1)
Опубликована: Янв. 1, 2025
Mitochondrial
quality
control
(MQC)
mechanisms
–
including
biogenesis,
dynamics,
mitophagy,
proteostasis,
the
unfolded
protein
response,
and
mitochondrial-derived
vesicles
play
critical
roles
in
development
of
atherosclerosis.
Dysregulation
these
processes
can
lead
to
mitochondrial
dysfunction,
subsequently
initiation
a
pathological
cascade
characterized
by
oxidative
stress,
chronic
inflammation,
accumulation
lipids
within
arterial
walls.
Specifically,
ROS
overproduction
redox
state
imbalance
are
key
molecular
aspects
that
exacerbate
damage,
create
self-perpetuating
cycle
cellular
injury
disease
progression.
Emerging
therapeutic
strategies
targeting
modulation
MQC
have
promise
attenuating
atherosclerotic
progression
restoring
balance
fusion
fission
enhancing
clearance
damaged
mitochondria,
improving
homeostasis.
Advancing
understanding
regulators
interaction
networks
pathways
might
facilitate
precision-targeted
therapies.
However,
substantial
challenges
persist
translating
insights
into
clinical
applications.
This
review
explores
relationship
between
atherosclerosis,
focusing
on
associated
potential
avenues
for
intervention.
Biomedicines,
Год журнала:
2023,
Номер
11(6), С. 1564 - 1564
Опубликована: Май 28, 2023
Mitochondrial
dysfunction
is
an
important
cellular
hallmark
of
aging
and
neurodegeneration.
Platelets
are
a
useful
model
to
study
the
systemic
manifestations
mitochondrial
dysfunction.
To
evaluate
age
dependence
parameters,
citrate
synthase
activity,
respiratory
chain
complex
oxygen
consumption
kinetics
were
assessed.
The
effect
cognitive
impairment
was
examined
by
comparing
parameters
in
healthy
individuals
those
with
neuropsychiatric
disease.
found
significant
negative
slope
age-dependence
for
both
activity
individual
enzymes
(citrate
II)
respiration
intact
platelets
(routine
respiration,
maximum
capacity
electron
transport
system,
rate
after
I
inhibition).
However,
there
no
difference
age-related
changes
between
without
impairment.
These
findings
highlight
potential
measuring
as
means
assess
results
indicate
that
drugs
interventions
targeting
may
have
slow
down
or
eliminate
certain
neurodegenerative
processes.
holds
promise
biomarker
aging,
irrespective
degree
