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Peroxisome deficient invertebrate and vertebrate animal models.
[zellweger syndrome]
Although
peroxisomes
are
ubiquitous
organelles
in
all
animal
species
,
their
importance
for
the
functioning
of
tissues
and
organs
remains
largely
unresolved
.
Because
peroxins
are
essential
for
the
biogenesis
of
peroxisomes
,
an
obvious
approach
to
investigate
their
physiological
role
is
to
inactivate
a
Pex
gene
or
to
suppress
its
translation
.
This
has
been
performed
in
mice
but
also
in
more
primitive
organisms
including
D
.
melanogaster
,
C
.
elegans
,
and
D
.
rerio
,
and
the
major
findings
and
abnormalities
in
these
models
will
be
highlighted
.
Although
peroxisomes
are
generally
not
essential
for
embryonic
development
and
organogenesis
,
a
generalized
inactivity
of
peroxisomes
affects
lifespan
and
posthatching
/
postnatal
growth
,
proving
that
peroxisomal
metabolism
is
necessary
for
the
normal
maturation
of
these
organisms
.
Strikingly
,
despite
the
wide
variety
of
model
organisms
,
corresponding
tissues
are
affected
including
the
central
nervous
system
and
the
testis
.
By
inactivating
peroxisomes
in
a
cell
type
selective
way
in
the
brain
of
mice
,
it
was
also
demonstrated
that
peroxisomes
are
necessary
to
prevent
neurodegeneration
.
As
these
peroxisome
deficient
model
organisms
recapitulate
pathologies
of
patients
affected
with
peroxisomal
diseases
,
their
further
analysis
will
contribute
to
the
elucidation
of
still
elusive
pathogenic
mechanisms
.
Diseases
Validation
Diseases presenting
"deficient model organisms recapitulate pathologies"
symptom
zellweger syndrome
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