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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
.
