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Association of mitochondrial letm1 with epileptic seizures.
[wolf-hirschhorn syndrome]
Leucine
zipper-
EF-
hand
containing
transmembrane
protein
1
(
Letm
1
)
is
a
mitochondrial
protein
that
is
associated
with
seizure
attacks
in
Wolf-
Hirschhorn
syndrome
.
This
study
aimed
to
investigate
the
expression
pattern
of
Letm
1
in
patients
with
temporal
lobe
epilepsy
(
TLE
)
and
pilocarpine-induced
rat
model
of
epilepsy
,
and
to
determine
if
altered
Letm
1
leads
to
mitochondrial
dysfunction
and
increased
susceptibility
to
seizures
.
Using
immunohistochemical
,
immunofluorescent
,
western
blotting
,
and
transmission
electron
microscopic
methods
,
we
have
found
that
Letm
1
was
significantly
decreased
in
TLE
patients
,
and
gradually
decreased
in
experimental
rats
from
1
to
7
days
after
onset
of
seizures
.
Letm
1
knock-down
by
a
lentivirus
bearing
LV-Letm
1
-
sh
resulted
in
mitochondrial
swelling
and
decreased
expression
of
Letm
1
target
protein
mitochondrially
encoded
cytochrome
B
(
MT-CYB
)
.
Behavioral
study
revealed
that
inhibition
of
Letm
1
caused
early
onset
of
the
first
seizure
,
increased
seizure
frequency
,
and
duration
.
However
,
administration
of
Letm
1
homolog
nigericin
failed
to
prevent
epilepsy
.
These
results
indicate
that
inhibition
of
Letm
1
and
mitochondrial
dysfunctions
contributes
to
the
development
of
epileptic
seizures
.
Appropriate
Letm
1
level
may
be
critical
for
maintaining
normal
neuronal
functions
.
