Rare Diseases Symptoms Automatic Extraction
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Current understanding on the pathogenesis of polyglutamine diseases.
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Polyglutamine
(
polyQ
)
diseases
are
a
family
of
neurodegenerative
disorders
including
Huntington
's
disease
,
spinobulbar
muscular
atrophy
,
dentatorubral-pallidoluysian
atrophy
and
several
spinocerebellar
ataxias
.
polyQ
diseases
are
caused
by
abnormal
expansion
of
CAG
repeats
in
certain
genes
.
The
expanded
CAG
repeats
are
then
translated
into
a
series
of
abnormally
expanded
polyQ
tracts
.
Such
polyQ
tracts
may
induce
misfolding
of
the
disease-causing
proteins
.
The
present
review
mainly
focuses
on
the
common
characteristics
of
the
pathogenesis
of
these
polyQ
diseases
,
including
conformational
transition
of
proteins
and
its
influence
on
the
function
of
these
proteins
,
the
correlation
between
decreased
ability
of
proteolysis
and
late-onset
polyQ
diseases
,
and
the
relationship
between
wide
expression
of
disease-causing
proteins
and
selective
neuronal
death
.
