Rare Diseases Symptoms Automatic Extraction
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Solubilized cystatin C amyloid is cytotoxic to cultured human cerebrovascular smooth muscle cells.
[hereditary cerebral hemorrhage with amyloidosis]
Cerebral
amyloid
angiopathy
(
CAA
)
is
characterized
by
the
accumulation
of
amyloid
within
arteries
of
the
cerebral
cortex
and
leptomeninges
.
This
condition
is
age
related
,
especially
prevalent
in
Alzheimer
's
disease
(
AD
)
and
the
main
feature
of
certain
hereditary
disorders
.
The
vascular
smooth
muscle
cells
(
VSMC
)
appear
to
play
a
vital
role
in
the
development
of
CAA
and
have
been
found
to
produce
the
amyloid
beta
precursor
protein
(
AbetaPP
)
and
process
it
to
Abeta
the
major
component
of
most
CAA
amyloid
.
Moreover
,
synthesized
Abeta
has
proven
to
be
toxic
to
cerebral
VSMC
in
culture
possibly
explaining
the
disintegration
and
disappearance
of
the
muscle
cells
from
affected
cerebral
blood
vessels
seen
in
CAA
.
An
aggressive
and
extremely
rare
form
of
CAA
,
known
as
Hereditary
Cerebral
Hemorrhage
With
Amyloidosis
-
Icelandic
Type
(
HCHWA
-
I
)
,
exhibits
this
withdrawal
of
VSMC
as
amyloid
accumulates
in
the
vessel
wall
.
However
,
the
amyloid
in
HCHWA
-
I
is
made
from
a
variant
of
cystatin
C
(
L
68
Q
)
instead
of
the
more
common
Abeta
.
To
evaluate
possible
cytotoxicity
in
this
condition
solubilized
cystatin
C
amyloid
extracted
from
HCHWA
-
I
leptomeninges
was
applied
to
cerebral
smooth
muscle
cells
in
culture
and
was
found
to
kill
the
cells
.
