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Mitochondrial and inflammatory changes in sporadic Inclusion Body Myositis.
[inclusion body myositis]
Sporadic
Inclusion
Body
Myositis
(
sIBM
)
is
the
most
common
late
onset
muscle
disease
causing
progressive
weakness
.
In
light
of
the
lack
of
effective
treatment
,
we
investigated
potential
causes
underlying
muscle
wasting
.
We
hypothesised
that
accumulation
of
mitochondrial
respiratory
deficiency
in
muscle
fibres
may
lead
to
fibre
atrophy
and
degeneration
,
contributing
to
muscle
mass
reduction
.
Histochemical
and
immunohistochemical
analyses
were
performed
on
muscle
biopsies
from
16
sIBM
patients
to
detect
activity
of
mitochondrial
enzymes
and
expression
of
mitochondrial
respiratory
chain
proteins
along
with
inflammatory
markers
respectively
.
Mitochondrial
DNA
mutations
were
assessed
in
single
muscle
fibres
using
Real-time
PCR
.
W
e
identified
respiratory
-
deficient
fibres
at
different
stages
of
mitochondrial
dysfunction
,
with
downregulated
expression
of
complex
I
of
mitochondrial
respiratory
chain
being
the
initial
feature
.
We
detected
mitochondrial
DNA
rearrangements
in
the
majority
of
individual
respiratory
-
deficient
muscle
fibres
.
There
was
a
strong
correlation
between
number
of
T
lymphocytes
and
macrophages
residing
in
muscle
tissue
and
the
abundance
of
respiratory
-
deficient
fibres
.
Moreover
,
we
found
that
respiratory
-
deficient
muscle
fibres
were
more
likely
to
be
atrophic
compared
to
respiratory
-normal
counterparts
.
Our
findings
suggest
that
mitochondrial
dysfunction
has
a
role
in
sIBM
progression
.
A
strong
correlation
between
the
severity
of
inflammation
,
degree
of
mitochondrial
changes
and
atrophy
implicated
existence
of
a
mechanistic
link
between
these
three
parameters
.
We
propose
a
role
for
inflammatory
cells
in
the
initiation
of
mitochondrial
DNA
damage
,
which
when
accumulated
,
causes
respiratory
dysfunction
,
fibre
atrophy
and
ultimately
degeneration
of
muscle
fibres
.
Diseases
Validation
Diseases presenting
"mitochondrial changes"
symptom
inclusion body myositis
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