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The human peroxisome in health and disease: the story of an oddity becoming a vital organelle.
[zellweger syndrome]
Since
the
first
report
by
Rhodin
in
1954
,
our
knowledge
on
mammalian
microbodies
/
peroxisomes
has
known
several
periods
.
An
initial
two
decades
period
(
1954
-
1973
)
has
contributed
to
the
biochemical
individualisation
of
peroxisomes
as
a
new
class
of
subcellular
organelles
(
de
Duve
,
1965
)
.
The
corresponding
research
period
failed
to
define
a
clear
role
of
mammalian
peroxisomes
in
vital
functions
and
intermediary
metabolism
,
explaining
why
feeling
that
peroxisomes
might
be
in
the
human
cell
oddities
has
prevailed
during
several
decades
.
The
period
standing
from
1973
to
nowadays
has
progressively
removed
this
cell
oddity
view
of
peroxisomes
by
highlighting
vital
function
and
metabolic
role
of
peroxisomes
in
health
and
disease
along
with
genetic
and
metabolic
regulation
of
peroxisomal
protein
content
,
organelle
envelope
formation
and
protein
signal
targeting
mechanisms
.
Research
on
peroxisomes
and
their
response
to
various
drugs
and
metabolites
,
dietary
and
physiological
conditions
has
also
played
a
key
role
in
the
discovery
of
peroxisome
proliferator
activated
receptors
(
PPARs
)
belonging
to
the
nuclear
hormone
receptor
superfamily
and
for
which
impact
in
science
and
medicine
goes
now
by
far
beyond
that
of
the
peroxisomes
.
Diseases
Validation
Diseases presenting
"known several periods. an initial two decades"
symptom
zellweger syndrome
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