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A random Abstract
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Measurement of peroxisomal fatty acid beta-oxidation in cultured human skin fibroblasts.
[neonatal adrenoleukodystrophy]
One
of
the
main
functions
of
mammalian
peroxisomes
is
the
beta
-oxidation
of
a
variety
of
fatty
acids
and
fatty
acid
derivatives
,
including
very
long
-chain
fatty
acids
.
Oxidation
of
these
fatty
acids
is
deficient
in
a
number
of
different
peroxisomal
disorders
,
including
the
disorders
of
peroxisome
biogenesis
(
Zellweger
syndrome
,
neonatal
adrenoleukodystrophy
and
infantile
Refsum
disease
)
,
X-
linked
adrenoleukodystrophy
and
a
number
of
other
disorders
of
peroxisomal
beta
-oxidation
of
known
and
unknown
aetiology
.
Accurate
measurement
of
peroxisomal
fatty
acid
oxidation
is
of
utmost
importance
for
correct
postnatal
and
prenatal
diagnosis
of
these
disorders
.
In
this
paper
we
describe
a
straightforward
and
accurate
assay
method
to
measure
the
beta
-oxidation
of
palmitic
acid
(
C
16
:
0
)
,
hexacosanoic
acid
(
C
2
6
:
0
)
and
pristanic
acid
in
intact
fibroblasts
.
