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Ribosome-binding proteins Mdm38 and Mba1 display overlapping functions for regulation of mitochondrial translation.
[wolf-hirschhorn syndrome]
Biogenesis
of
respiratory
chain
complexes
depends
on
the
expression
of
mitochondrial
-encoded
subunits
.
Their
synthesis
occurs
on
membrane-associated
ribosomes
and
is
probably
coupled
to
their
membrane
insertion
.
Defects
in
expression
of
mitochondrial
translation
products
are
among
the
major
causes
of
mitochondrial
disorders
.
Mdm
38
is
related
to
Letm
1
,
a
protein
affected
in
Wolf-
Hirschhorn
syndrome
patients
.
Like
Mba
1
and
Oxa
1
,
Mdm
38
is
an
inner
membrane
protein
that
interacts
with
ribosomes
and
is
involved
in
respiratory
chain
biogenesis
.
We
find
that
simultaneous
loss
of
Mba
1
and
Mdm
38
causes
severe
synthetic
defects
in
the
biogenesis
of
cytochrome
reductase
and
cytochrome
oxidase
.
These
defects
are
not
due
to
a
compromised
membrane
binding
of
ribosomes
but
the
consequence
of
a
mis-regulation
in
the
synthesis
of
Cox
1
and
cytochrome
b
.
Cox
1
expression
is
restored
by
replacing
Cox
1
-
specific
regulatory
regions
in
the
mRNA
.
We
conclude
,
that
Mdm
38
and
Mba
1
exhibit
overlapping
regulatory
functions
in
translation
of
selected
mitochondrial
mRNAs
.
Diseases
Validation
Diseases presenting
"selected mitochondrial mrnas"
symptom
wolf-hirschhorn syndrome
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