Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
Sporadic inclusion-body myositis: A degenerative muscle disease associated with aging, impaired muscle protein homeostasis and abnormal mitophagy.
[inclusion body myositis]
Sporadic
inclusion-body
myositis
(
s-
IBM
)
is
the
most
common
degenerative
muscle
disease
in
which
aging
appears
to
be
a
key
risk
factor
.
In
this
review
we
focus
on
several
cellular
molecular
mechanisms
responsible
for
multiprotein
aggregation
and
accumulations
within
s-
IBM
muscle
fibers
,
and
their
possible
consequences
.
Those
include
mechanisms
leading
to
:
a
)
accumulation
in
the
form
of
aggregates
within
the
muscle
fibers
,
of
several
proteins
,
including
amyloid-β
42
and
its
oligomers
,
and
phosphorylated
tau
in
the
form
of
paired
helical
filaments
,
and
we
consider
their
putative
detrimental
influence
;
and
b
)
protein
misfolding
and
aggregation
,
including
evidence
of
abnormal
myoproteostasis
,
such
as
increased
protein
transcription
,
inadequate
protein
disposal
,
and
abnormal
posttranslational
modifications
of
proteins
.
Pathogenic
importance
of
our
recently
demonstrated
abnormal
mitophagy
is
also
discussed
.
The
intriguing
phenotypic
similarities
between
s-
IBM
muscle
fibers
and
the
brains
of
Alzheimer
and
Parkinson
's
disease
patients
,
the
two
most
common
neurodegenerative
diseases
associated
with
aging
,
are
also
discussed
.
This
article
is
part
of
a
Special
Issue
entitled
:
Neuromuscular
Diseases
:
Pathology
and
Molecular
Pathogenesis
.
Diseases
Validation
Diseases presenting
"muscle fibers"
symptom
epidermolysis bullosa simplex
focal myositis
inclusion body myositis
pyomyositis
systemic capillary leak syndrome
You can validate or delete this automatically detected symptom
Validate the Symptom
Delete the Symptom