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Pendrin overexpression affects cell volume recovery, intracellular pH and chloride concentration after hypotonicity-induced cell swelling.
[pendred syndrome]
The
pendrin
(
SLC
26
A
4
or
PDS
)
gene
is
responsible
,
when
mutated
,
for
the
Pendred
syndrome
,
a
recessive
disorder
characterized
by
sensorineural
hearing
loss
often
accompanied
by
thyroid
dysfunctions
.
Pendrin
protein
is
an
anion
exchanger
and
we
focused
on
a
still
unexplored
function
that
it
might
play
in
view
of
its
importance
in
the
inner
ear
:
Cl
(
-
)
fluxes
regulation
during
cellular
volume
control
.
We
challenged
HEK-
293
Phoenix
cells
over-expressing
wild
type
pendrin
(
PDS
HEK
cells
)
together
with
the
EYFP
(
Enhanced
Yellow
Fluorescent
Protein
)
or
over-expressing
the
EYFP
alone
(
control
HEK
cells
)
with
hypo-osmolar
solutions
.
Taking
advantage
of
the
confocal
optical
sectioning
we
measured
the
cell
volume
.
In
addition
,
we
determined
the
intracellular
pH
and
chloride
concentration
with
fluorescent
probes
(
EYFP
and
seminaphthorhodafluor-
5
F
,
SNARF-
5
F
)
.
Consequently
,
we
could
estimate
simultaneously
Cl
(
-
)
fluxes
,
cellular
volume
and
intracellular
pH
variations
.
Cl
(
-
)
movements
markedly
differed
between
PDS
and
control
HEK
cells
upon
hypotonic
shock
and
are
accompanied
by
an
attenuation
of
the
swelling
induced
pH
drop
in
PDS
HEK
cells
.
The
contemporary
measurements
of
the
three
variables
not
yet
reported
in
living
cells
,
allowed
to
assess
a
possible
influence
of
pendrin
upregulation
in
volume
homeostasis
and
evidenced
its
participation
to
Cl
(
-
)
fluxes
.