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[Roles of cyclooxygenase 2/2', 3'-cyclic nucleotide3' phosphohydrolase in the oligodendrocyte apoptosis in heroin-induced spongiform leucoencephalopathy].
[canavan disease]
To
investigate
the
regulation
of
cyclooxygenase
(
Cox
)
-
2
/
2
'
,
3
'
-
cyclic
nucleotide
3
'
phosphohydrolase
(
CNPase
)
on
the
oligodendrocyte
apoptosis
in
the
pathogenesis
of
the
heroin-induced
spongiform
leucoencephalopathy
(
HSLE
)
.
Samples
of
frontal
lobe
,
cerebellum
,
and
corpus
callosum
were
obtained
from
the
brains
during
autopsy
of
4
HSLE
patients
and
5
patients
who
died
of
diseases
other
than
cerebral
diseases
(
controls
)
and
underwent
light
microscopy
and
electron
microscopy
.
Immunocytochemistry
was
carried
out
to
detect
the
expression
of
myelin
basic
protein
(
MBP
)
,
caspase-
3
,
COX
-
2
,
and
CNPase
protein
.
Apoptosis
was
examined
by
TUNEL
staining
.
Widespread
demyelination
was
seen
in
the
white
matter
of
the
frontal
lobe
,
cerebellum
,
and
corpus
callosum
of
the
HSLE
cases
,
most
severely
in
cerebellum
.
In
he
HSLE
group
,
the
levels
of
caspase-
3
and
COX
-
2
expression
were
significantly
higher
,
and
the
level
of
CNPase
was
significantly
lower
than
those
of
the
control
group
(
all
P
<
0
.
05
)
.
Widespread
demyelination
in
the
white
matter
is
a
prevailing
pathological
change
of
HSLE
.
Oligodendrocyte
apoptosis
is
one
of
the
causes
of
HSLE
.
The
upregulation
of
COX
-
2
and
downregulation
of
CNPase
may
contribute
to
the
pathogenesis
.
Diseases
Validation
Diseases presenting
"corpus callosum"
symptom
adrenomyeloneuropathy
alexander disease
cadasil
canavan disease
classical phenylketonuria
cohen syndrome
gm1 gangliosidosis
hirschsprung disease
hydrocephalus with stenosis of the aqueduct of sylvius
kabuki syndrome
kallmann syndrome
krabbe disease
monosomy 21
neonatal adrenoleukodystrophy
oligodontia
proteus syndrome
pyruvate dehydrogenase deficiency
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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