Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Role of polymorphonuclear leukocyte-derived serine proteinases in defense against Actinobacillus actinomycetemcomitans.
[papillon-lefèvre syndrome]
Periodontitis
is
a
chronic
destructive
infection
of
the
tooth-supportive
tissues
,
which
is
caused
by
pathogenic
bacteria
such
as
Actinobacillus
actinomycetemcomitans
.
A
severe
form
of
periodontitis
is
found
in
Papillon-
Lefèvre
syndrome
(
PLS
)
,
an
inheritable
disease
caused
by
loss
-of-function
mutations
in
the
cathepsin
C
gene
.
Recently
,
we
demonstrated
that
these
patients
lack
the
activity
of
the
polymorphonuclear
leukocyte
(
PMN
)
-
derived
serine
proteinases
elastase
,
cathepsin
G
,
and
proteinase
3
.
In
the
present
study
we
identified
possible
pathways
along
which
serine
proteinases
may
be
involved
in
the
defense
against
A
.
actinomycetemcomitans
.
Serine
proteinases
are
capable
to
convert
the
PMN-derived
hCAP-
18
into
LL-
37
,
an
antimicrobial
peptide
with
activity
against
A
.
actinomycetemcomitans
.
We
found
that
the
PMNs
of
PLS
patients
released
lower
levels
of
LL-
37
.
Furthermore
,
because
of
their
deficiency
in
serine
proteases
,
the
PMNs
of
PLS
patients
were
incapable
of
neutralizing
the
leukotoxin
produced
by
this
pathogen
,
which
resulted
in
increased
cell
damage
.
Finally
,
the
capacity
of
PMNs
from
PLS
patients
to
kill
A
.
actinomycetemcomitans
in
an
anaerobic
environment
,
such
as
that
found
in
the
periodontal
pocket
,
seemed
to
be
reduced
.
Our
report
demonstrates
a
mechanism
that
suggests
a
direct
link
between
an
inheritable
defect
in
PMN
functioning
and
difficulty
in
coping
with
a
periodontitis
-associated
pathogen
.
Diseases
Validation
Diseases presenting
"chronic destructive infection of the tooth-supportive tissues"
symptom
papillon-lefèvre syndrome
You can validate or delete this automatically detected symptom
Validate the Symptom
Delete the Symptom