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Metabolic functions of peroxisomes in health and disease.
[zellweger syndrome]
Peroxisomes
are
subcellular
organelles
which
are
present
in
virtually
every
eukaryotic
cell
and
catalyze
a
large
number
of
metabolic
functions
.
The
importance
of
peroxisomes
for
humans
is
stressed
by
the
existence
of
a
large
group
of
genetic
diseases
in
which
either
the
biogenesis
of
peroxisomes
is
impaired
or
one
of
its
metabolic
functions
.
Thanks
to
the
work
on
Zellweger
syndrome
which
is
the
prototype
of
the
group
of
peroxisomal
disorders
,
much
has
been
learned
about
the
metabolism
and
biogenesis
of
peroxisomes
in
humans
.
These
metabolic
functions
include
:
(
1
.
)
fatty
acid
beta
-oxidation
;
(
2
.
)
etherphospholipid
biosynthesis
;
(
3
.
)
fatty
acid
alpha-oxidation
,
and
(
4
.
)
glyoxylate
detoxification
.
Since
peroxisomes
lack
a
citric
acid
cycle
and
a
respiratory
chain
,
peroxisomes
are
relatively
helpless
organelles
which
rely
heavily
on
their
cross-talk
with
other
subcellular
organelles
in
order
to
metabolize
the
end
products
of
metabolism
as
generated
in
peroxisomes
.
The
metabolic
functions
of
peroxisomes
in
humans
will
be
briefly
described
in
this
review
with
emphasis
on
the
cross-talk
with
other
subcellular
organelles
as
well
as
the
peroxisomal
disorders
in
which
one
or
more
peroxisomal
functions
are
impaired
.
Diseases
Validation
Diseases presenting
"large number"
symptom
acute rheumatic fever
adrenal incidentaloma
allergic bronchopulmonary aspergillosis
canavan disease
coats disease
cowden syndrome
dedifferentiated liposarcoma
dracunculiasis
epidermolysis bullosa simplex
fabry disease
familial mediterranean fever
gm1 gangliosidosis
heparin-induced thrombocytopenia
hereditary cerebral hemorrhage with amyloidosis
hirschsprung disease
kindler syndrome
legionellosis
malignant atrophic papulosis
neuralgic amyotrophy
phenylketonuria
pleomorphic liposarcoma
primary effusion lymphoma
scrub typhus
severe combined immunodeficiency
triple a syndrome
waldenström macroglobulinemia
well-differentiated liposarcoma
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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