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Filipin recognizes both GM1 and cholesterol in GM1 gangliosidosis mouse brain.
[gm1 gangliosidosis]
Filipin
is
an
antibiotic
polyene
widely
used
as
a
histochemical
marker
for
cholesterol
.
We
previously
reported
cholesterol
/
filipin-
positive
staining
in
brain
of
β-galactosidase
(
β-gal
)
knockout
(
(
-
/
-
)
)
mice
(
GM
1
gangliosidosis
)
.
The
content
and
distribution
of
cholesterol
and
gangliosides
was
analyzed
in
plasma
membrane
(
PM
)
and
microsomal
(
MS
)
fractions
from
whole-
brain
tissue
of
15
week
-old
control
(
β-gal
(
+
/
-
)
)
and
GM
1
gangliosidosis
(
β-gal
(
-
/
-
)
)
mice
.
Total
ganglioside
content
(
μg
sialic
acid
/
mg
protein
)
was
3
-
fold
and
7
-
fold
greater
in
the
PM
and
MS
fractions
,
respectively
,
in
βgal
(
-
/
-
)
mice
than
in
βgal
(
+
/
-
)
mice
.
GM
1
content
was
30
-
fold
and
50
-
fold
greater
in
the
PM
and
MS
fractions
,
respectively
.
In
contrast
,
unesterified
cholesterol
content
(
μg
/
mg
protein
)
was
similar
in
the
PM
and
the
MS
fractions
of
the
βgal
(
-
/
-
)
and
βgal
(
+
/
-
)
mice
.
Filipin
is
known
to
bind
to
various
sterol
derivatives
and
phospholipids
on
thin
-layer
chromatograms
.
Biochemical
evidence
is
presented
showing
that
filipin
also
binds
to
GM
1
with
an
affinity
similar
to
that
for
cholesterol
,
with
a
corresponding
fluorescent
reaction
.
Our
data
suggest
that
the
GM
1
storage
seen
in
the
β-gal
(
-
/
-
)
mouse
contributes
to
the
filipin
ultraviolet
fluorescence
observed
in
GM
1
gangliosidosis
brain
.
The
data
indicate
that
in
addition
to
cholesterol
,
filipin
can
also
be
useful
for
detecting
GM
1
.
Diseases
Validation
Diseases presenting
"thin-layer chromatograms"
symptom
gm1 gangliosidosis
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