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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
"severe combined immunodeficiency"
symptom
achondroplasia
alpha-thalassemia
child syndrome
cholangiocarcinoma
junctional epidermolysis bullosa
krabbe disease
omenn syndrome
severe combined immunodeficiency
wiskott-aldrich syndrome
This symptom has already been validated