Autoinflammatorische
Erkrankungen
zeichnen
sich
durch
entzündliche
Manifestationen
in
verschiedenen
Organsystemen
aus,
wobei
wiederkehrende
Fieberschübe,
muskuloskeletale
Beschwerden,
gastrointestinale
und
kutane
Symptome,
begleitet
von
serologischen
Entzündungszeichen,
häufig
auftreten.
Die
autoinflammatorischen
umfassen
seltene
monogenetische
Entitäten
sowie
multifaktorielle/polygene
Krankheiten,
die
mit
variabler
Symptomatik
im
Laufe
der
Zeit
manifestieren
können.
Beispiele
für
autoinflammatorische
sind
das
familiäre
Mittelmeerfieber
(FMF),
Cryopyrin-assoziierte
periodische
Syndrom
(CAPS),
TNF(Tumor-Nekrose-Faktor)-Rezeptor-assoziierte
(TRAPS)
neu
beschriebene
VEXAS-Syndrom.
Bei
den
nichtmonogenetisch
determinierten
wichtigsten
Vertreter
Erwachsenenalter
adulte
Form
Still-Erkrankung
(AOSD)
Schnitzler-Syndrom,
bei
denen
eher
eine
polygenetische
Suszeptibilität
epigenetische
Faktoren
Rolle
spielen.
International Journal of Molecular Sciences,
Год журнала:
2024,
Номер
25(9), С. 5001 - 5001
Опубликована: Май 3, 2024
Abdominal
aortic
aneurysm
(AAA)
has
been
recognized
as
a
serious
chronic
inflammatory
degenerative
disease
in
recent
years.
At
present,
there
is
no
other
effective
intervention
except
surgical
treatment
for
AAA.
With
the
aging
of
human
population,
its
incidence
increasing
year
by
year,
posing
threat
to
health.
Modern
studies
suggest
that
vascular
response
core
process
AAA
occurrence
and
development.
Inflammasome,
multiprotein
complex
located
cytoplasm,
mediates
expression
various
cytokines
like
interleukin
(IL)-1β
IL-18,
thus
plays
pivotal
role
inflammation
regulation.
Therefore,
inflammasome
may
exert
crucial
influence
on
progression
This
article
reviews
some
mechanism
investigate
then
summarizes
several
potential
drugs
targeting
AAA,
aiming
provide
new
ideas
clinical
prevention
beyond
methods.
Cells,
Год журнала:
2024,
Номер
13(16), С. 1365 - 1365
Опубликована: Авг. 16, 2024
The
NLRP3
inflammasome
is
a
critical
component
of
the
innate
immune
response.
activation
tightly
controlled
process
involving
an
initial
priming
to
express
NLRP3,
pro-IL-1
β,
and
pro-IL-18,
followed
by
signal.
precise
mechanism
not
fully
understood
due
diverse
range
activators,
yet
it
effectively
orchestrates
caspase-1,
which
subsequently
triggers
release
proinflammatory
cytokines
IL-1β
IL-18.
dysregulation
can
lead
variety
inflammatory
diseases,
highlighting
its
significant
role
in
response
disease
pathogenesis.
divided
into
three
domains:
PYD,
NACHT,
LRR
domains.
This
review
focuses
on
domain
detailing
structural
characteristics,
function
pathogen
sensing,
degradation
process,
involvement
auto-inhibition
activation.
Additionally,
we
discuss
impact
mutations
within
found
atypical
Cryopyrin-Associated
Periodic
Syndromes
(CAPS),
clinical
relevance
this
domain.
Proceedings of the National Academy of Sciences,
Год журнала:
2025,
Номер
122(8)
Опубликована: Фев. 20, 2025
Death
domain
fold
(DDF)
superfamily
proteins
are
critically
important
players
in
pathways
of
cell
death
and
inflammation.
DDFs
often
essential
scaffolding
domains
receptors,
adaptors,
or
effectors
these
by
mediating
homo-
hetero-oligomerization
including
helical
filament
assembly.
At
the
downstream
ends
pathways,
effector
oligomerization
brings
enzyme
into
proximity
for
their
dimerization
activation.
Hundreds
structures
have
been
solved.
However,
a
comprehensive
understanding
is
lacking.
In
this
article,
we
report
curation
DDF
structural
atlas
as
public
website
(deathdomain.org)
deduce
common
distinct
principles
DDF-mediated
among
four
families
(death
DD,
DED,
caspase
recruitment
CARD,
pyrin
PYD).
We
further
annotate
genome-wide
based
on
AlphaFold-predicted
models
protein
sequences.
These
studies
reveal
mechanistic
rules
widely
distributed
superfamily.
bioRxiv (Cold Spring Harbor Laboratory),
Год журнала:
2024,
Номер
unknown
Опубликована: Сен. 6, 2024
Inflammation
has
an
essential
role
in
healing.
However,
over-active
inflammation
disrupts
normal
cellular
functions
and
can
be
life-threatening
when
not
resolved.
The
NLRP3
inflammasome,
a
component
of
the
innate
immune
system,
is
intracellular
multiprotein
complex
that
senses
stress-associated
signals,
and,
for
this
reason
promising
therapeutic
target
treating
unresolved,
pathogenic
inflammation.
Alternative
splicing
