Rare Diseases Symptoms Automatic Extraction
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A random Abstract
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Our Team
Of Omenn and mice.
[omenn syndrome]
Omenn
syndrome
(
OS
)
is
a
peculiar
immunodeficiency
in
which
profound
T
and
B
cell
defects
are
associated
with
severe
autoimmune
manifestations
.
Although
the
molecular
and
biochemical
bases
of
OS
have
been
elucidated
,
the
mechanisms
leading
to
T
cell
infiltration
of
peripheral
tissues
such
as
skin
and
gut
still
remain
unsolved
.
Two
murine
models
with
hypomorphic
mutations
in
rag
genes
reproducing
OS
features
and
a
murine
model
of
lymphopenia
-derived
autoimmunity
with
similar
immunopathology
were
recently
described
.
The
aim
of
this
review
is
to
integrate
clues
regarding
the
roles
of
impaired
thymic
development
and
lymphopenia
into
the
pathogenesis
of
autoimmunity
.