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Increase of ceramide monohexoside and dipalmitoyl glycerophospholipids in the brain of Zellweger syndrome.
[zellweger syndrome]
The
lipid
composition
and
molecular
species
of
phospholipids
were
examined
in
the
brain
of
a
patient
with
Zellweger
syndrome
(
ZS
)
,
and
were
compared
with
those
of
control
infants
.
In
the
cerebral
gray
matter
of
the
ZS
patient
,
the
amounts
of
ceramide
monohexoside
and
cholesterol
ester
were
larger
than
those
of
controls
.
By
contrast
,
the
amount
of
ceramide
monohexoside
in
the
white
matter
was
smaller
in
the
ZS
patient
than
that
in
the
age-matched
control
.
Although
the
amount
of
phosphatidylcholine
(
PC
)
plus
phosphatidylserine
(
PS
)
was
the
same
,
dipalmitoyl
PC
and
PS
were
increased
in
both
the
gray
and
white
matter
of
the
ZS
cerebrum
.
These
alterations
in
the
molecular
species
of
brain
lipids
may
play
crucial
roles
in
the
pathogenesis
of
ZS
.
