Rare Diseases Symptoms Automatic Extraction
Home
A random Abstract
Our Project
Our Team
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
"cerebral regions"
symptom
hydrocephalus with stenosis of the aqueduct of sylvius
neonatal adrenoleukodystrophy
You can validate or delete this automatically detected symptom
Validate the Symptom
Delete the Symptom