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Keratinocyte antiviral response to Poly(dA:dT) stimulation and papillomavirus infection in a canine model of X-linked severe combined immunodeficiency.
[severe combined immunodeficiency]
X-
linked
severe
combined
immunodeficiency
(
XSCID
)
is
caused
by
a
genetic
mutation
within
the
common
gamma
chain
(
γc
)
,
an
essential
component
of
the
cytokine
receptors
for
interleukin
(
IL
)
-
2
,
IL
-
4
,
IL
-
7
,
IL
-
9
,
IL
-
15
,
and
IL
-
21
.
XSCID
patients
are
most
commonly
treated
with
bone
marrow
transplants
(
BMT
)
to
restore
systemic
immune
function
.
However
,
BMT-XSCID
humans
and
dogs
remain
at
an
increased
risk
for
development
of
cutaneous
papillomavirus
(
PV
)
infections
and
their
associated
neoplasms
,
most
typically
cutaneous
papillomas
.
Since
basal
keratinocytes
are
the
target
cell
for
the
initial
PV
infection
,
we
wanted
to
determine
if
canine
XSCID
keratinocytes
have
a
diminished
antiviral
cytokine
response
to
poly
(
dA
:
dT
)
and
canine
papillomavirus-
2
(
CPV-
2
)
upon
initial
infection
.
We
performed
quantitative
RT-PCR
for
antiviral
cytokines
and
downstream
interferon
stimulated
genes
(
ISG
)
on
poly
(
dA
:
dT
)
stimulated
and
CPV-
2
infected
monolayer
keratinocyte
cultures
derived
from
XSCID
and
normal
control
dogs
.
We
found
that
XSCID
keratinocytes
responded
similarly
to
poly
(
dA
:
dT
)
as
normal
keratinocytes
by
upregulating
antiviral
cytokines
and
ISGs
.
CPV-
2
infection
of
both
XSCID
and
normal
keratinocytes
did
not
result
in
upregulation
of
antiviral
cytokines
or
ISGs
at
2
,
4
,
or
6
days
post
infection
.
These
data
suggest
that
the
antiviral
response
to
initial
PV
infection
of
basal
keratinocytes
is
similar
between
XSCID
and
normal
patients
,
and
is
not
the
likely
source
for
the
remaining
immunodeficiency
in
XSCID
patients
.
Diseases
Validation
Diseases presenting
"immunodeficiency"
symptom
adrenal incidentaloma
allergic bronchopulmonary aspergillosis
cushing syndrome
dracunculiasis
hirschsprung disease
hodgkin lymphoma, classical
homocystinuria without methylmalonic aciduria
kabuki syndrome
legionellosis
malignant atrophic papulosis
oculocutaneous albinism
omenn syndrome
papillon-lefèvre syndrome
primary effusion lymphoma
primary hyperoxaluria type 1
pyomyositis
severe combined immunodeficiency
sneddon syndrome
werner syndrome
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
This symptom has already been validated