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Peroxisomes in human health and disease: metabolic pathways, metabolite transport, interplay with other organelles and signal transduction.
[zellweger syndrome]
Peroxisomes
play
a
key
role
in
human
physiology
as
exemplified
by
the
devastating
consequences
of
a
defect
in
peroxisome
biogenesis
as
observed
in
patients
affected
by
Zellweger
syndrome
.
The
main
metabolic
functions
of
peroxisomes
in
humans
include
:
(
1
)
fatty
acid
beta
-oxidation
;
(
2
)
etherphospholipid
synthesis
;
(
3
)
bile
acid
synthesis
;
(
4
)
fatty
acid
alpha-oxidation
,
and
(
5
)
glyoxylate
detoxification
.
Since
peroxisomes
lack
a
citric
acid
cycle
and
respiratory
chain
like
mitochondria
do
,
metabolism
in
peroxisomes
requires
continued
cross-talk
with
other
organelles
,
notably
mitochondria
and
the
endoplasmic
reticulum
in
order
to
allow
continued
metabolism
of
the
products
generated
by
peroxisomes
.
Many
of
the
metabolites
which
require
peroxisomes
for
homeostasis
,
are
involved
in
signal
transduction
pathways
.
These
include
the
primary
bile
acids
;
platelet
activating
factor
;
plasmalogens
,
N-
acylglycines
and
N-
acyltaurines
;
docosahexaenoic
acid
as
well
as
multiple
prostanoids
.
The
current
state
of
knowledge
in
this
area
will
be
discussed
in
this
review
.
