Journal of Inherited Metabolic Disease,
Год журнала:
2024,
Номер
48(1)
Опубликована: Июнь 14, 2024
Abstract
Mitochondria
are
dynamic
cellular
organelles
with
complex
roles
in
metabolism
and
signalling.
Primary
mitochondrial
disorders
a
group
of
approximately
400
monogenic
arising
from
pathogenic
genetic
variants
impacting
structure,
ultrastructure
and/or
function.
Amongst
these
disorders,
defects
lipid
biosynthesis,
especially
the
unique
membrane
cardiolipin,
biology
an
emerging
characterised
by
clinical
heterogeneity,
but
recurrent
features
including
cardiomyopathy,
encephalopathy,
neurodegeneration,
neuropathy
3‐methylglutaconic
aciduria.
This
review
discusses
synthesis
membrane,
contact
site
cristae
organising
system
(MICOS),
dynamics
trafficking,
associated
each
processes.
We
highlight
overlapping
functions
proteins
involved
biosynthesis
protein
import
into
mitochondria,
pointing
to
overarching
coordination
synchronisation
functions.
also
focuses
on
interactions
between
mitochondria
other
organelles,
namely
endoplasmic
reticulum,
peroxisomes,
lysosomes
droplets.
signpost
that
may
explain
observation
secondary
dysfunction
heterogeneous
pathological
Disruption
organellar
ultimately
impairs
homeostasis
organismal
health,
highlighting
central
role
human
health
disease.
Signal Transduction and Targeted Therapy,
Год журнала:
2024,
Номер
9(1)
Опубликована: Май 15, 2024
Abstract
Mitochondria,
with
their
intricate
networks
of
functions
and
information
processing,
are
pivotal
in
both
health
regulation
disease
progression.
Particularly,
mitochondrial
dysfunctions
identified
many
common
pathologies,
including
cardiovascular
diseases,
neurodegeneration,
metabolic
syndrome,
cancer.
However,
the
multifaceted
nature
elusive
phenotypic
threshold
dysfunction
complicate
our
understanding
contributions
to
diseases.
Nonetheless,
these
complexities
do
not
prevent
mitochondria
from
being
among
most
important
therapeutic
targets.
In
recent
years,
strategies
targeting
have
continuously
emerged
transitioned
clinical
trials.
Advanced
intervention
such
as
using
healthy
replenish
or
replace
damaged
mitochondria,
has
shown
promise
preclinical
trials
various
Mitochondrial
components,
mtDNA,
mitochondria-located
microRNA,
associated
proteins
can
be
potential
agents
augment
function
immunometabolic
diseases
tissue
injuries.
Here,
we
review
current
knowledge
pathophysiology
concrete
examples
We
also
summarize
treat
perspective
dietary
supplements
targeted
therapies,
well
translational
situation
related
pharmacology
agents.
Finally,
this
discusses
innovations
applications
transplantation
an
advanced
promising
treatment.
Advanced Drug Delivery Reviews,
Год журнала:
2024,
Номер
210, С. 115342 - 115342
Опубликована: Май 24, 2024
Chronic
non-healing
wounds
persist
as
a
substantial
burden
for
healthcare
systems,
influenced
by
factors
such
aging,
diabetes,
and
obesity.
In
contrast
to
the
traditionally
pro-regenerative
emphasis
of
therapies,
recognition
immune
system
integral
role
in
wound
healing
has
significantly
grown,
instigating
an
approach
shift
towards
immunological
processes.
Thus,
this
review
explores
process,
highlighting
engagement
system,
delving
into
behaviors
innate
adaptive
cells
chronic
scenarios.
Moreover,
article
investigates
biomaterial-based
strategies
modulation
elucidating
how
adjustment
their
physicochemical
properties
or
synergistic
combination
with
other
agents
drugs,
proteins
mesenchymal
stromal
can
effectively
modulate
different
cells.
Finally
various
based
on
synthetic
biological
nanostructures,
including
extracellular
vesicles,
finely
tune
natural
immunomodulators
therapeutic
nanocarriers
promising
biophysical
properties.
International Journal of Molecular Sciences,
Год журнала:
2023,
Номер
24(18), С. 13835 - 13835
Опубликована: Сен. 8, 2023
Mitophagy
is
crucial
for
maintaining
mitochondrial
quality.
However,
its
assessment
in
vivo
challenging.
The
endosomal-lysosomal
system
a
more
accessible
pathway
through
which
subtypes
of
extracellular
vesicles
(EVs),
also
contain
constituents,
are
released
disposal.
inclusion
components
into
EVs
occurs
the
setting
mild
damage
and
during
impairment
lysosomal
function.
By
releasing
mitochondrial-derived
(MDVs),
cells
limit
unload
damage-associated
molecular
patterns
with
proinflammatory
activity.
Both
positive
negative
effects
on
recipient
have
been
described.
Whether
this
due
to
production
other
than
those
containing
mitochondria,
such
as
MDVs,
holding
specific
biological
functions
currently
unknown.
Evidence
existence
different
MDV
has
produced.
their
characterization
not
always
pursued,
would
be
relevant
exploring
dynamics
quality
control
health
disease.
Furthermore,
classification
may
instrumental
understanding
roles
promoting
implementation
biomarkers
clinical
studies.
In
addition
to
their
pivotal
roles
in
energy
storage
and
expenditure,
adipose
tissues
play
a
crucial
part
the
secretion
of
bioactive
molecules,
including
peptides,
lipids,
metabolites,
extracellular
vesicles,
response
physiological
stimulation
metabolic
stress.
These
secretory
factors,
through
autocrine
paracrine
mechanisms,
regulate
various
processes
within
tissues.
include
adipogenesis,
glucose
lipid
metabolism,
inflammation,
adaptive
thermogenesis,
all
which
are
essential
for
maintenance
balance
functionality
tissue
micro-environment.
A
subset
these
adipose-derived
factors
can
enter
circulation
target
distant
appetite,
cognitive
function,
insulin
sensitivity,
gluconeogenesis,
cardiovascular
remodeling,
exercise
capacity.
this
review,
we
highlight
role
signaling
pathways
modulating
homeostasis.
Furthermore,
delve
into
alterations
both
content
under
pathological
conditions,
shedding
light
on
potential
pharmacological
treatment
strategies
related
diseases.
