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Molecular species of phospholipids with very long chain fatty acids in skin fibroblasts of Zellweger syndrome.
[zellweger syndrome]
The
ratio
of
C
26
:
0
/
C
22
:
0
fatty
acids
in
patient
lipids
is
widely
accepted
as
a
critical
clinical
criterion
of
peroxisomal
diseases
,
such
as
Zellweger
syndrome
and
X-
linked
adrenoleukodystrophy
(
X-
ALD
)
.
However
,
phospholipid
molecular
species
with
very
long
chain
fatty
acids
(
VLCFA
)
have
not
been
precisely
characterized
.
In
the
present
study
,
the
structures
of
such
molecules
in
fibroblasts
of
Zellweger
syndrome
and
X-
ALD
were
examined
using
LC
-
ESI
-
MS
/
MS
analysis
.
In
fibroblasts
from
Zellweger
patients
,
a
large
number
of
VLCFA-containing
molecular
species
were
detected
in
several
phospholipid
classes
as
well
as
neutral
lipids
,
including
triacylglycerol
and
cholesteryl
esters
.
Among
these
lipids
,
phosphatidylcholine
showed
the
most
diversity
in
the
structures
of
VLCFA-containing
molecular
species
.
Some
VLCFA
possessed
longer
carbon
chains
and
/
or
larger
number
of
double
bonds
than
C
26
:
0
-
fatty
acid
(
FA
)
.
Similar
VLCFA
were
also
found
in
other
phospholipid
classes
,
such
as
phosphatidylethanolamine
and
phosphatidylserine
.
In
addition
,
VLCFA-containing
phospholipid
species
showed
some
differences
among
fibroblasts
from
Zellweger
patients
.
It
appears
that
phospholipids
with
VLCFA
,
with
or
without
double
bonds
,
as
well
as
C
26
:
0
-
FA
might
affect
cellular
functions
,
thus
leading
to
the
pathogenesis
of
peroxisomal
diseases
,
such
as
Zellweger
syndrome
and
X-
ALD
.
Diseases
Validation
Diseases presenting
"large number"
symptom
acute rheumatic fever
adrenal incidentaloma
allergic bronchopulmonary aspergillosis
canavan disease
coats disease
cowden syndrome
dedifferentiated liposarcoma
dracunculiasis
epidermolysis bullosa simplex
fabry disease
familial mediterranean fever
gm1 gangliosidosis
heparin-induced thrombocytopenia
hereditary cerebral hemorrhage with amyloidosis
hirschsprung disease
kindler syndrome
legionellosis
malignant atrophic papulosis
neuralgic amyotrophy
phenylketonuria
pleomorphic liposarcoma
primary effusion lymphoma
scrub typhus
severe combined immunodeficiency
triple a syndrome
waldenström macroglobulinemia
well-differentiated liposarcoma
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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