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Phosphatidyl ethanolamine with increased polyunsaturated fatty acids in compensation for plasmalogen defect in the Zellweger syndrome brain.
[zellweger syndrome]
To
elucidate
the
neuropathological
mechanism
of
Zellweger
syndrome
(
ZS
)
,
we
studied
changes
in
the
molecular
species
of
glycerophospholipids
in
the
cerebral
tissue
by
thin
-layer
chromatography
(
TLC
)
and
fast
atom
bombardment
mass
spectrometry
(
FABMS
)
.
First
,
we
estimated
the
amount
of
plasmalogens
by
TLC
.
Plasmalogen-
type
phosphatidyl
ethanolamine
(
PE
)
accounted
for
30
%
of
the
total
PE
in
the
control
brain
,
but
was
absent
in
the
ZS
brain
.
Plasmalogen-
type
phosphatidyl
choline
(
PC
)
was
undetectable
in
both
control
and
ZS
brains
.
Next
,
we
analyzed
plasmalogen-
type
PE
by
FABMS
.
Oleic
(
18
:
1
)
,
arachidonic
(
20
:
4
)
and
docosapentanoic
(
22
:
5
)
acids
were
present
in
the
control
gray
matter
,
but
not
in
the
ZS
gray
matter
.
In
compensation
for
the
defect
of
plasmalogen
,
the
level
of
diacyl
PE
with
polyunsaturated
fatty
acids
,
20
:
4
,
22
:
4
,
22
:
5
and
22
:
6
,
was
higher
in
the
ZS
brain
than
that
in
the
control
brain
.
These
results
indicate
an
alteration
in
the
molecular
species
of
PE
,
which
may
cause
abnormal
neural
membrane
fluidity
and
excessive
vulnerability
to
oxygen
stress
.
Diseases
Validation
Diseases presenting
"thin-layer chromatography"
symptom
canavan disease
krabbe disease
neonatal adrenoleukodystrophy
zellweger syndrome
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