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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
.
Diseases
Validation
Diseases presenting
"central nervous system"
symptom
22q11.2 deletion syndrome
adrenomyeloneuropathy
alexander disease
aniridia
aromatase deficiency
canavan disease
child syndrome
classical phenylketonuria
congenital toxoplasmosis
cowden syndrome
cushing syndrome
cystinuria
dracunculiasis
erdheim-chester disease
fabry disease
gm1 gangliosidosis
hereditary cerebral hemorrhage with amyloidosis
hirschsprung disease
hodgkin lymphoma, classical
kabuki syndrome
kallmann syndrome
kindler syndrome
krabbe disease
lamellar ichthyosis
legionellosis
liposarcoma
malignant atrophic papulosis
monosomy 21
neonatal adrenoleukodystrophy
phenylketonuria
proteus syndrome
scrub typhus
severe combined immunodeficiency
sneddon syndrome
triple a syndrome
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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