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On the presence of C2-ceramide in mammalian tissues: possible relationship to etherphospholipids and phosphorylation by ceramide kinase.
[zellweger syndrome]
C
(
2
)
-
ceramide
(
N-
acetyl-sphingenine
)
is
often
used
as
an
analog
to
study
ceramide-mediated
cellular
processes
.
According
to
Lee
et
al
.
[
J
.
Biol
.
Chem
.
271
(
1996
)
,
209
-
217
]
,
C
(
2
)
-
ceramide
is
formed
by
an
acetyl
transfer
from
platelet
-activating
factor
(
PAF
,
1
-
O-
alkyl-
2
-
acetyl-
sn
-glycero-
3
-
phosphocholine
)
to
sphingenine
.
To
substantiate
these
unconfirmed
findings
,
we
(
i
)
developed
a
method
to
quantify
C
(
2
)
-
ceramide
and
(
ii
)
analyzed
C
(
2
)
-
ceramide
levels
in
Pex
5
(
-
/
-
)
mice
,
a
model
for
Zellweger
syndrome
,
in
which
the
synthesis
of
ether
lipids
such
as
PAF
is
impaired
.
The
presence
of
C
(
2
)
-
ceramide
could
be
established
in
brain
(
+
/
-
10
pmol
/
g
)
and
liver
(
+
/
-
25
pmol
/
g
)
from
control
mice
,
and
was
approximately
5000
-
fold
less
than
the
main
long
-chain
ceramide
species
.
In
Pex
5
(
-
/
-
)
mice
,
C
(
2
)
-
ceramide
levels
did
not
differ
significantly
compared
to
control
tissues
.
Given
the
presence
of
a
ceramide
kinase
in
mammals
,
phosphorylation
of
C
(
2
)
-
ceramide
by
human
ceramide
kinase
(
HsCERK
)
was
tested
.
C
(
2
)
-
ceramide
appears
to
be
a
good
substrate
when
albumin
is
used
as
carrier
.
In
CHO
cells
overexpressing
HsCERK
,
phosphorylation
of
exogenously
added
C
(
2
)
-
ceramide
could
also
be
demonstrated
.
Our
data
indicate
that
C
(
2
)
-
ceramide
is
present
in
mammalian
tissues
and
can
be
converted
to
C
(
2
)
-
ceramide-
1
-
phosphate
,
in
addition
to
other
documented
metabolic
alterations
,
but
does
not
seem
to
be
linked
to
ether
lipid
metabolism
.
Diseases
Validation
Diseases presenting
"but does not seem to be linked to ether lipid metabolism"
symptom
zellweger syndrome
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