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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
"brain tissue"
symptom
22q11.2 deletion syndrome
alexander disease
cadasil
classical phenylketonuria
congenital toxoplasmosis
gm1 gangliosidosis
hereditary cerebral hemorrhage with amyloidosis
homocystinuria without methylmalonic aciduria
hydrocephalus with stenosis of the aqueduct of sylvius
phenylketonuria
pyruvate dehydrogenase deficiency
sneddon syndrome
wolf-hirschhorn syndrome
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