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A quantitative proteomic approach to identify significantly altered protein networks in the serum of patients with lymphangioleiomyomatosis (LAM).
[lymphangioleiomyomatosis]
Lymphangioleiomyomatosis
(
LAM
)
is
a
rare
and
progressive
cystic
lung
condition
affecting
approximately
3
.
4
-
7
.
5
/
million
women
,
with
an
average
lag
time
between
symptom
onset
and
diagnosis
of
upwards
of
4
years
.
The
aim
of
this
work
was
to
identify
altered
proteins
in
LAM
serum
which
may
be
potential
biomarkers
of
disease
.
Serum
from
LAM
patient
volunteers
and
healthy
control
volunteers
were
pooled
and
analysis
carried
out
using
quantitative
4
-
plex
iTRAQ
technology
.
Differentially
expressed
proteins
were
validated
using
ELISAs
and
pathway
analysis
was
carried
out
using
Ingenuity
Pathway
Analysis
.
Fourteen
proteins
were
differentially
expressed
in
LAM
serum
compared
to
control
serum
(
p
<
0
.
05
)
.
Further
screening
validated
the
observed
differences
in
extracellular
matrix
remodelling
proteins
including
fibronectin
(
30
%
decrease
in
LAM
,
p
=
0
.
03
)
,
von
Willebrand
Factor
(
40
%
reduction
in
LAM
,
p
=
0
.
03
)
and
Kallikrein
III
(
25
%
increase
in
LAM
,
p
=
0
.
03
)
.
Pathway
networks
elucidated
the
relationships
between
the
ECM
and
cell
trafficking
in
LAM
.
This
study
was
the
first
to
highlight
an
imbalance
in
networks
important
for
remodelling
in
LAM
,
providing
a
set
of
novel
potential
biomarkers
.
These
understandings
may
lead
to
a
new
effective
treatment
for
LAM
in
the
future
.
Diseases
Validation
Diseases presenting
"further screening"
symptom
cadasil
lymphangioleiomyomatosis
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