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Myelin peroxisomes - essential organelles for the maintenance of white matter in the nervous system.
[x-linked adrenoleukodystrophy]
Peroxisomes
are
cellular
compartments
primarily
associated
with
lipid
metabolism
.
Most
cell
types
,
including
nervous
system
cells
,
harbor
several
hundred
of
these
organelles
.
The
importance
of
peroxisomes
for
central
nervous
system
white
matter
is
evidenced
by
a
variety
of
human
peroxisomal
disorders
with
neurological
impairment
frequently
involving
the
white
matter
.
Moreover
,
the
most
frequent
childhood
white
matter
disease
,
X-
linked
adrenoleukodystrophy
,
is
a
peroxisomal
disorder
.
During
the
past
decade
advances
in
imaging
techniques
have
enabled
the
identification
of
peroxisomes
within
the
myelin
sheath
,
especially
close
to
nodes
of
Ranvier
.
Although
the
function
of
myelin
peroxisomes
is
not
solved
yet
on
molecular
level
,
recently
acquired
knowledge
suggests
a
central
role
for
these
organelles
in
axo-glial
metabolism
.
This
review
focuses
on
the
biology
of
myelin
peroxisomes
as
well
as
on
the
pathology
of
myelin
and
myelinated
axons
that
is
observed
as
a
consequence
of
partial
or
complete
peroxisomal
dysfunction
in
the
brain
.