Journal of Cerebral Blood Flow & Metabolism,
Journal Year:
2025,
Volume and Issue:
unknown
Published: March 13, 2025
Demyelination
is
a
common
feature
of
neuroinflammatory
and
degenerative
diseases
the
central
nervous
system
(CNS),
such
as
multiple
sclerosis
(MS).
It
often
linked
to
disruptions
in
intercellular
communication,
bioenergetics
metabolic
balance
accompanied
by
mitochondrial
dysfunction
cells
oligodendrocytes,
neurons,
astrocytes,
microglia.
Although
current
MS
treatments
focus
on
immunomodulation,
they
fail
stop
or
reverse
demyelination’s
progression.
Recent
advancements
highlight
exchange
promising
therapeutic
target,
with
potential
restore
homeostasis,
enhance
promote
myelin
repair.
With
this
review
we
will
provide
insights
into
CNS
decoupling,
focusing
role
demyelinating
conditions.
We
then
discuss
emerging
cell-free
biotherapies
exploring
transferring
mitochondria
via
biogenic
carriers
like
extracellular
vesicles
(EVs)
synthetic
liposomes,
aimed
at
enhancing
function
support
for
Lastly,
address
key
challenges
clinical
application
these
strategies
future
directions
optimize
biotherapies.
The
field
hold
promise
restoring
repair,
potentially
transforming
landscape
diseases.
Hepatology,
Journal Year:
2024,
Volume and Issue:
unknown
Published: April 29, 2024
Mitochondria
are
intracellular
organelles
responsible
for
energy
production,
glucose
and
lipid
metabolism,
cell
death,
proliferation,
innate
immune
response.
highly
dynamic
that
constantly
undergo
fission,
fusion,
trafficking,
as
well
degradation
biogenesis.
Mitochondrial
dysfunction
has
been
implicated
in
a
variety
of
chronic
liver
diseases
including
alcohol-associated
disease,
metabolic
dysfunction–associated
steatohepatitis,
HCC.
In
this
review,
we
provide
detailed
overview
mitochondrial
dynamics,
mitophagy,
DNA–mediated
response,
how
dysregulation
these
processes
affects
the
pathogenesis
disease
dynamics
response
may
thereby
represent
an
attractive
therapeutic
target
ameliorating
Journal of Inherited Metabolic Disease,
Journal Year:
2024,
Volume and Issue:
47(5), P. 903 - 916
Published: May 24, 2024
Mitochondria
carry
out
essential
functions
for
the
cell,
including
energy
production,
various
biosynthesis
pathways,
formation
of
co-factors
and
cellular
signalling
in
apoptosis
inflammation.
The
functionality
mitochondria
requires
import
about
900-1300
proteins
from
cytosol
baker's
yeast
Saccharomyces
cerevisiae
human
cells,
respectively.
vast
majority
these
pass
outer
membrane
a
largely
unfolded
state
through
translocase
mitochondrial
(TOM)
complex.
Subsequently,
specific
protein
translocases
sort
precursor
into
inner
membranes,
intermembrane
space
matrix.
Premature
folding
proteins,
defects
or
reduction
potential
across
can
cause
stalling
precursors
at
apparatus.
Consequently,
translocon
is
clogged
non-imported
accumulate
which
turn
leads
to
proteotoxic
stress
eventually
cell
death.
To
prevent
such
situations,
quality
control
mechanisms
remove
TOM
channel.
highly
conserved
ubiquitin-proteasome
system
plays
critical
role
this
process.
Thus,
surveillance
via
complex
involves
coordinated
activity
mitochondria-localized
cytosolic
cell.
ACS Nano,
Journal Year:
2024,
Volume and Issue:
18(37), P. 25372 - 25404
Published: Sept. 3, 2024
Mitochondria,
pivotal
organelles
governing
cellular
biosynthesis,
energy
metabolism,
and
signal
transduction,
maintain
dynamic
equilibrium
through
processes
such
as
biogenesis,
fusion,
fission,
mitophagy.
Growing
evidence
implicates
mitochondrial
dysfunction
in
a
spectrum
of
respiratory
diseases
including
acute
lung
injury/acute
distress
syndrome,
bronchial
asthma,
pulmonary
fibrosis,
chronic
obstructive
disease,
cancer.
Consequently,
identifying
methods
capable
ameliorating
damaged
function
is
crucial
for
the
treatment
diseases.
Extracellular
vesicles
(EVs),
nanosized
membrane
released
by
cells
into
extracellular
space,
facilitate
intercellular
communication
transferring
bioactive
substances
or
signals
between
organs.
Recent
studies
have
identified
abundant
components
within
specific
subsets
EVs,
termed
(mitoEVs),
whose
contents
compositions
vary
with
disease
progression.
Moreover,
mitoEVs
demonstrated
reparative
functions
injured
recipient
cells.
However,
comprehensive
understanding
currently
lacking,
limiting
their
clinical
translation
prospects.
This
Review
explores
classification,
functional
cargo,
biological
effects
mitoEVs,
focus
on
role
Emphasis
placed
potential
markers
innovative
therapeutic
strategies
diseases,
offering
fresh
insights
mechanistic
drug
development
various
disorders.
Signal Transduction and Targeted Therapy,
Journal Year:
2025,
Volume and Issue:
10(1)
Published: March 3, 2025
Abstract
Mitochondrial
diseases
represent
one
of
the
most
prevalent
and
debilitating
categories
hereditary
disorders,
characterized
by
significant
genetic,
biological,
clinical
heterogeneity,
which
has
driven
development
field
engineered
mitochondria.
With
growing
recognition
pathogenic
role
damaged
mitochondria
in
aging,
oxidative
inflammatory
diseases,
cancer,
application
expanded
to
those
non-hereditary
contexts
(sometimes
referred
as
mitochondria-related
diseases).
Due
their
unique
non-eukaryotic
origins
endosymbiotic
relationship,
are
considered
highly
suitable
for
gene
editing
intercellular
transplantation,
remarkable
progress
been
achieved
two
promising
therapeutic
strategies—mitochondrial
artificial
mitochondrial
transfer
(collectively
this
review)
over
past
decades.
Here,
we
provide
a
comprehensive
review
mechanisms
recent
advancements
applications,
alongside
concise
summary
potential
implications
supporting
evidence
from
preclinical
studies.
Additionally,
an
emerging
potentially
feasible
approach
involves
ex
vivo
editing,
followed
selection
holds
overcome
limitations
such
reduced
operability
introduction
allogeneic
thereby
broadening
applicability
Science Advances,
Journal Year:
2025,
Volume and Issue:
11(14)
Published: April 4, 2025
Mitochondrial
DNA
(mtDNA)
is
exposed
to
multiple
insults
produced
by
normal
cellular
function.
Upon
mtDNA
replication
stress,
the
mitochondrial
genome
transfers
endosomes
for
degradation.
Using
proximity
biotinylation,
we
found
that
stress
leads
rewiring
of
proteome,
increasing
mitochondria’s
association
with
lysosomal
and
vesicle-related
proteins.
Among
these,
retromer
complex,
particularly
VPS35,
plays
a
pivotal
role
extracting
components.
The
promotes
formation
mitochondrial-derived
vesicles
shuttled
lysosomes.
mtDNA,
however,
directly
shuttles
recycling
organelle
in
BAX-dependent
manner.
Moreover,
using
Drosophila
model
carrying
long
deletion
on
(ΔmtDNA),
ΔmtDNA
activates
specific
transcriptome
profile
counteract
damage.
Here,
Vps35
expression
restores
homoplasmy
alleviates
associated
defects.
Hence,
demonstrate
existence
previously
unknown
quality
control
mechanism
matrix
essential
lysosomes
turnover
relieve
