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Immunocytochemical localization of acyl-CoA oxidase in the rat central nervous system.
[neonatal adrenoleukodystrophy]
Peroxisomal
beta
-oxidation
,
consisting
of
four
steps
catalysed
by
an
acyl-
CoA
oxidase
,
a
multifunctional
protein
and
a
thiolase
,
is
responsible
for
the
shortening
of
a
variety
of
lipid
compounds
.
The
first
reaction
of
this
pathway
is
catalysed
by
a
FAD-containing
acyl-
CoA
oxidase
,
three
isotypes
of
which
have
been
so
far
recognised
.
Among
these
,
straight-chain
acyl-
CoA
oxidase
(
ACOX
)
acts
on
long
and
very
long
chain
fatty
acids
,
prostaglandins
and
some
xenobiotics
.
We
investigated
ACOX
localisation
by
means
of
a
sensitive
,
tyramide
based
,
immunocytochemical
technique
,
thus
obtaining
a
complete
distribution
atlas
of
the
enzyme
in
adult
rat
CNS
.
Granular
immunoreaction
product
was
found
in
the
cytoplasm
of
neuronal
and
glial
cells
,
both
in
the
perikarya
and
in
the
cell
processes
.
ACOX
immunoreactive
neurons
were
present
to
variable
extent
,
in
either
forebrain
or
hindbrain
areas
.
Specifically
,
the
strongest
signal
was
detected
in
the
pallidum
,
septum
,
red
nucleus
,
reticular
formation
,
nuclei
of
the
cranial
nerves
,
and
motoneurons
of
the
spinal
cord
.
We
then
compared
the
ACOX
immunoreactivity
pattern
with
our
previous
distribution
maps
of
other
peroxisomal
enzymes
in
the
adult
rat
brain
.
While
ACOX
appeared
to
colocalise
with
catalase
in
the
majority
of
cerebral
regions
,
some
differences
with
respect
to
d-amino
acid
oxidase
were
noted
.
These
observations
support
the
hypothesis
of
heterogeneous
peroxisomal
populations
in
the
nervous
tissue
.
The
wide
distribution
of
the
enzyme
in
the
brain
is
consistent
with
the
severe
and
generalised
neurological
alterations
characterising
the
peroxisomal
disorder
caused
by
ACOX
deficiency
(
pseudo-
neonatal
adrenoleukodystrophy
)
.
Diseases
Validation
Diseases presenting
"first reaction of this pathway"
symptom
neonatal adrenoleukodystrophy
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