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Pendrin: the thyrocyte apical membrane iodide transporter?
[pendred syndrome]
In
the
thyroid
,
the
transport
of
iodide
from
the
extracellular
space
to
the
follicular
lumen
requires
two
steps
:
the
transport
in
the
cell
at
the
basal
side
and
in
the
lumen
at
the
apical
side
.
The
first
step
is
mediated
by
the
Na
(
+
)
/
I
(
-
)
symporter
(
NIS
)
.
In
most
reviews
and
textbooks
,
the
second
step
is
presented
as
mediated
by
pendrin
.
In
this
review
,
we
analyze
this
assumption
.
There
are
several
arguments
supporting
the
concept
that
indeed
pendrin
plays
an
important
role
in
thyroid
physiology
.
However
,
biochemical
,
clinical
and
histological
data
on
the
thyroid
of
a
patient
with
Pendred
syndrome
do
not
suggest
an
essential
role
in
iodide
transport
,
which
is
corroborated
by
the
lack
of
a
thyroid
phenotype
in
pendrin
knockout
mice
.
Experiments
in
vivo
and
in
vitro
on
polarized
and
unpolarized
cells
show
that
iodide
is
transported
transport
of
iodide
at
the
apex
of
the
thyroid
cell
.
Moreover
,
ectopic
expression
of
pendrin
in
transfected
non-
thyroid
cells
is
capable
of
mediating
iodide
efflux
.
It
is
concluded
that
pendrin
may
participate
in
the
iodide
efflux
into
thyroid
lumen
but
not
as
the
unique
transporter
.
Moreover
,
another
role
of
pendrin
in
mediating
Cl
(
-
)
/
HCO
(
3
)
(
-
)
exchange
and
controlling
luminal
pH
is
suggested
.
Diseases
Validation
Diseases presenting
"indeed pendrin"
symptom
pendred syndrome
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