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Tissue transglutaminase colocalizes with extracellular matrix proteins in cerebral amyloid angiopathy.
[hereditary cerebral hemorrhage with amyloidosis]
Cerebral
amyloid
angiopathy
(
CAA
)
is
a
key
histopathological
hallmark
of
Alzheimer
's
disease
(
AD
)
and
hereditary
cerebral
hemorrhage
with
amyloidosis
of
the
Dutch
type
(
HCHWA-D
)
.
CAA
is
characterized
by
amyloid-
beta
(
Aβ
)
depositions
and
remodeling
of
the
extracellular
matrix
(
ECM
)
in
brain
vessels
and
plays
an
important
role
in
the
development
and
progression
of
both
AD
and
HCHWA-D
.
Tissue
transglutaminase
(
tTG
)
modulates
the
ECM
by
molecular
cross-linking
of
ECM
proteins
.
Here
,
we
investigated
the
distribution
pattern
,
cellular
source
,
and
activity
of
tTG
in
CAA
in
control
,
AD
,
and
HCHWA-D
cases
.
We
observed
increased
tTG
immunoreactivity
and
colocalization
with
Aβ
in
the
vessel
wall
in
early
stage
CAA
,
whereas
in
later
CAA
stages
,
tTG
and
its
cross-links
were
present
in
halos
enclosing
the
Aβ
deposition
.
In
CAA
,
tTG
and
its
cross-links
at
the
abluminal
side
of
the
vessel
were
demonstrated
to
be
either
of
astrocytic
origin
in
parenchymal
vessels
,
of
fibroblastic
origin
in
leptomeningeal
vessels
,
and
of
endothelial
origin
at
the
luminal
side
of
the
deposited
Aβ
.
Furthermore
,
the
ECM
proteins
fibronectin
and
laminin
colocalized
with
the
tTG-
positive
halos
surrounding
the
deposited
Aβ
in
CAA
.
However
,
we
observed
that
in
situ
tTG
activity
was
present
throughout
the
vessel
wall
in
late
stage
CAA
.
Together
,
our
data
suggest
that
tTG
and
its
activity
might
play
a
differential
role
in
the
development
and
progression
of
CAA
,
possibly
evolving
from
direct
modulation
of
Aβ
aggregation
to
cross-linking
of
ECM
proteins
resulting
in
ECM
restructuring
.
Diseases
Validation
Diseases presenting
"late stage"
symptom
familial mediterranean fever
hereditary cerebral hemorrhage with amyloidosis
lymphangioleiomyomatosis
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