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Association of LETM1 and MRPL36 contributes to the regulation of mitochondrial ATP production and necrotic cell death.
[wolf-hirschhorn syndrome]
Leucine
zipper
/
EF
hand
-containing
transmembrane-
1
(
LETM
1
)
is
a
mitochondrial
inner
membrane
protein
that
was
first
identified
in
Wolf-
Hirschhorn
syndrome
,
and
was
deleted
in
nearly
all
patients
with
the
syndrome
.
LETM
1
encodes
for
the
human
homologue
of
yeast
Mdm
38
p
,
which
is
a
mitochondria-shaping
protein
of
unclear
function
.
Here
,
we
describe
LETM
1
-
mediated
regulation
of
mitochondrial
ATP
production
and
biogenesis
.
We
show
that
LETM
1
overexpression
can
induce
necrotic
cell
death
in
HeLa
cells
,
in
which
LETM
1
reduces
mitochondrial
biogenesis
and
ATP
production
.
LETM
1
acts
as
an
anchor
protein
and
associates
with
mitochondrial
ribosome
protein
L
36
.
Adenovirus-mediated
overexpression
of
LETM
1
reduced
mitochondrial
mass
and
expression
of
many
mitochondrial
proteins
.
LETM
1
-
mediated
inhibition
of
mitochondrial
biogenesis
enhanced
glycolytic
ATP
supply
and
activated
protein
kinase
B
activity
and
cell
survival
signaling
.
The
expression
levels
of
LETM
1
were
significantly
increased
in
multiple
human
cancer
tissues
compared
with
normals
.
These
data
suggest
that
LETM
1
serves
as
an
anchor
protein
for
complex
formation
with
the
mitochondrial
ribosome
and
regulates
mitochondrial
biogenesis
.
The
increased
expression
of
LETM
1
in
human
cancer
suggests
that
dysregulation
of
LETM
1
is
a
key
feature
of
tumorigenesis
.
Diseases
Validation
Diseases presenting
"complex formation with the mitochondrial ribosome"
symptom
wolf-hirschhorn syndrome
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