Rare Diseases Symptoms Automatic Extraction
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A random Abstract
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Selective extraction and effective separation of galactosylsphingosine (psychosine) and glucosylsphingosine from other glycosphingolipids in pathological tissue samples.
[krabbe disease]
To
facilitate
the
study
of
the
chemical
pathology
of
galactosylsphingosine
(
psychosine
,
GalSph
)
in
Krabbe
disease
and
glucosylsphingosine
(
GlcSph
)
in
Gaucher
disease
,
we
have
devised
a
facile
method
for
the
effective
separation
of
these
two
glycosylsphingosines
from
other
glycosphingolipids
(
GSLs
)
in
Krabbe
brain
and
Gaucher
spleen
samples
.
The
procedure
involves
the
use
of
acetone
to
selectively
extract
GalSph
and
GlcSph
,
respectively
,
from
Krabbe
brain
and
Gaucher
spleen
samples
.
Since
acetone
does
not
extract
other
GSLs
except
modest
amounts
of
galactosylceramide
,
sulfatide
,
and
glucosylceramide
,
the
positively
charged
GalSph
or
GlcSph
in
the
acetone
extract
can
be
readily
separated
from
other
GSLs
by
batchwise
cation-exchange
chromatography
using
a
Waters
Accell
Plus
CM
Cartridge
.
GalSph
or
GlcSph
enriched
by
this
simple
procedure
can
be
readily
analyzed
by
thin
-layer
chromatography
or
high
-performance
liquid
chromatography
.
Diseases
Validation
Diseases presenting
"high-performance liquid chromatography"
symptom
benign recurrent intrahepatic cholestasis
congenital adrenal hyperplasia
dentinogenesis imperfecta
krabbe disease
neonatal adrenoleukodystrophy
pendred syndrome
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