Research Square (Research Square),
Год журнала:
2024,
Номер
unknown
Опубликована: Май 10, 2024
Abstract
We
describe
eighteen
individuals
from
twelve
families
with
an
autosomal
recessive
neurodevelopmental
disorder
and
variable
leukodystrophy
harbouring
biallelic
variants
in
SUPV3L1.
SUPV3L1
encodes
the
RNA
helicase
SUV3
(also
known
as
SUPV3L1),
previous
studies
demonstrating
a
role
for
protein
part
of
mitochondrial
degradosome.
Patient
mutations
result
accumulation
double
stranded
RNAs
human
cells.
An
assessment
supv3l1
knock-out
zebrafish
confirmed
supv3l1
neurodevelopment,
gross
defects
identified
biogenesis
microglial
function.
Zebrafish
displayed
significant
activation
type
1
interferon
pathway,
which
was
supported
by
qPCR
blood
four
patients
mutations.
Altogether,
we
clinico-radiological
spectrum
associated
mutations,
that
loss
function
results
altered
biogenesis,
increased
RNA,
dysplastic
microglia
innate
immune
pathway.
Immunological Reviews,
Год журнала:
2023,
Номер
321(1), С. 246 - 262
Опубликована: Окт. 12, 2023
Summary
Cell
death
can
be
executed
through
distinct
subroutines.
PANoptosis
is
a
unique
inflammatory
cell
modality
involving
the
interactions
between
pyroptosis,
apoptosis,
and
necroptosis,
which
mediated
by
multifaceted
PANoptosome
complexes
assembled
via
integrating
components
from
other
modalities.
There
growing
interest
in
process
function
of
PANoptosis.
Accumulating
evidence
suggests
that
occurs
under
diverse
stimuli,
for
example,
viral
or
bacterial
infection,
cytokine
storm,
cancer.
Given
impact
across
disease
spectrum,
this
review
briefly
describes
relationships
highlights
key
molecules
formation
activation,
outlines
roles
diseases
together
with
potential
therapeutic
targeting.
We
also
discuss
important
concepts
pressing
issues
future
research.
Improved
understanding
its
mechanisms
crucial
identifying
novel
targets
strategies.
Molecular Biomedicine,
Год журнала:
2024,
Номер
5(1)
Опубликована: Апрель 22, 2024
Abstract
NLRP
inflammasomes
are
a
group
of
cytosolic
multiprotein
oligomer
pattern
recognition
receptors
(PRRs)
involved
in
the
pathogen-associated
molecular
patterns
(PAMPs)
and
danger-associated
(DAMPs)
produced
by
infected
cells.
They
regulate
innate
immunity
triggering
protective
inflammatory
response.
However,
despite
their
role,
aberrant
NLPR
inflammasome
activation
gain-of-function
mutations
sensor
proteins
occurrence
enhancement
non-communicating
autoimmune,
auto-inflammatory,
neurodegenerative
diseases.
In
last
few
years,
significant
advances
have
been
achieved
understanding
physiological
functions
mechanisms
activation,
as
well
therapeutics
that
target
activity
Here,
we
provide
latest
research
progress
on
inflammasomes,
including
NLRP1,
CARD8,
NLRP3,
NLRP6,
NLRP7,
NLRP2,
NLRP9,
NLRP10,
NLRP12
regarding
structural
assembling
features,
signaling
transduction
mechanisms.
Importantly,
highlight
associated
with
dysregulation
numerous
human
Overall,
summarize
discoveries
biology,
forming
role
health
diseases,
therapeutic
strategies
perspectives
for
future
studies
about
inflammasomes.
Frontiers in Immunology,
Год журнала:
2025,
Номер
15
Опубликована: Янв. 15, 2025
The
severity
of
spinal
cord
injury
(SCI)
is
closely
tied
to
pulmonary
function,
especially
in
cases
higher
SCI
levels.
Despite
this
connection,
the
underlying
pathological
mechanisms
lungs
post-SCI
are
not
well
understood.
Previous
research
has
established
a
connection
between
disrupted
sympathetic
regulation
and
splenocyte
apoptosis
high
thoracic
SCI,
leading
dysfunction.
aim
study
was
investigate
whether
mice
with
low-level
exhibit
increased
susceptibility
acute
lung
by
eliciting
systemic
inflammatory
responses
that
operate
independently
nervous
system.
Here,
we
employed
T9
contusion
exposed
aerosolized
lipopolysaccharide
(LPS)
simulate
inflammation
associated
respiratory
distress
syndrome
(ARDS).
Twenty-four
hours
post-LPS
exposure,
tissues
bronchoalveolar
lavage
(BAL)
fluid
were
analyzed.
LPS
markedly
induced
proinflammatory
gene
expression
(SAA3,
IRG1,
NLRP3,
IL-1beta,
MCP-1)
cytokine
release
(IL-1beta,
IL-6,
compared
controls,
indicating
an
exaggerated
response.
Infiltration
Ly6G/C
positive
neutrophils
macrophages
significantly
exposure.
Interestingly,
spleen
size
weight
did
differ
control
mice,
suggesting
alone
does
cause
atrophy.
Notably,
bone-marrow-derived
(BMDMs)
from
exhibited
hyper-responsiveness
LPS.
This
demonstrated
increase
immune
subsequent
underscoring
widespread
influence
post-SCI,
pronounced
specific
organs
like
lungs.
Advanced Healthcare Materials,
Год журнала:
2025,
Номер
unknown
Опубликована: Март 17, 2025
Abstract
During
the
bone
tissue
repair
process,
highly
dynamic
interactions
between
host
and
materials
hinder
precise,
stable,
sustained
immune
modulation.
Regulating
response
based
on
potential
mechanisms
of
macrophage
phenotypic
changes
may
represent
an
effective
strategy
for
promoting
healing.
This
study
successfully
constructs
a
co‐dispersed
pFe₃O₄‐MXene
nanosystem
by
loading
positively
charged
magnetite
(pFe₃O₄)
nanoparticles
onto
MXene
nanosheets
using
electrostatic
self‐assembly.
Subsequently,
this
work
fabricates
biomimetic
porous
scaffold
(PFM)
via
selective
laser
sintering,
which
exhibit
superior
magnetic
properties,
mechanical
performance,
hydrophilicity,
biocompatibility.
Further
investigations
demonstrate
that
PFM
could
precisely
remotely
modulate
polarization
toward
M2
phenotype
under
static
field,
significantly
enhancing
osteogenesis
angiogenesis.
Proteomic
analysis
reveal
upregulates
Arg2
expression,
mitochondrial
function
accelerating
oxidative
phosphorylation,
thereby
inducing
transition.
In
vivo
experiments
validated
scaffold's
regulatory
capacity
in
subcutaneous
cranial
defect
repairs
rats,
effectively
new
formation.
Overall,
modulation
targeting
metabolism
offers
novel
insights
material
design
engineering
regenerative
medicine.
Clinical Epigenetics,
Год журнала:
2025,
Номер
17(1)
Опубликована: Апрель 6, 2025
Abstract
Sepsis
is
a
syndrome
of
organ
dysfunction
caused
by
the
invasion
pathogenic
microorganisms.
In
clinical
practice,
patients
with
sepsis
are
prone
to
concurrent
acute
kidney
injury,
which
has
high
morbidity
and
mortality
rates.
Thus,
understanding
pathogenesis
sepsis-associated
injury
significant
importance.
Ferroptosis
an
iron-dependent
programmed
cell
death
pathway,
proved
play
critical
role
in
process
through
various
mechanisms.
Epigenetic
regulation
modulates
content
function
nucleic
acids
proteins
within
cells
modifications.
Its
impact
on
ferroptosis
garnered
increasing
attention;
however,
epigenetic
targeting
not
been
fully
elucidated.
Growing
evidence
suggests
that
can
modulate
complex
pathway
networks,
thereby
affecting
development
prognosis
injury.
This
paper
summarizes
regulatory
mechanisms
ferroptosis,
providing
new
insights
for
targeted
therapy
