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Peroxisome biogenesis in mammalian cells.
[zellweger syndrome]
To
investigate
peroxisome
assembly
and
human
peroxisome
biogenesis
disorders
(
PBDs
)
such
as
Zellweger
syndrome
,
thirteen
different
complementation
groups
(
CGs
)
of
Chinese
hamster
ovary
(
CHO
)
cell
mutants
defective
in
peroxisome
biogenesis
have
been
isolated
and
established
as
a
model
research
system
.
Successful
gene
-cloning
studies
by
a
forward
genetic
approach
utilized
a
rapid
functional
complementation
assay
of
CHO
cell
mutants
led
to
isolation
of
human
peroxin
(
PEX
)
genes
.
Search
for
pathogenic
genes
responsible
for
PBDs
of
all
14
CGs
is
now
completed
together
with
the
homology
search
by
screening
the
human
expressed
sequence
tag
database
using
yeast
PEX
genes
.
Peroxins
are
divided
into
three
groups
:
(
1
)
peroxins
including
Pex
3
p
,
Pex
16
p
,
and
Pex
19
p
,
are
responsible
for
peroxisome
membrane
biogenesis
via
classes
I
and
II
pathways
;
(
2
)
peroxins
that
function
in
matrix
protein
import
;
(
3
)
those
such
as
three
forms
of
Pex
11
p
,
Pex
11
pα
,
Pex
11
pβ
,
and
Pex
11
pγ
,
are
involved
in
peroxisome
proliferation
where
DLP
1
,
Mff
,
and
Fis
1
coordinately
function
.
In
membrane
assembly
,
Pex
19
p
forms
complexes
in
the
cytosol
with
newly
synthesized
PMPs
including
Pex
16
p
and
transports
them
to
the
receptor
Pex
3
p
,
whereby
peroxisomal
membrane
is
formed
(
Class
I
pathway
)
.
Pex
19
p
likewise
forms
a
complex
with
newly
made
Pex
3
p
and
translocates
it
to
the
Pex
3
p
receptor
,
Pex
16
p
(
Class
II
pathway
)
.
In
matrix
protein
import
,
newly
synthesized
proteins
harboring
peroxisome
targeting
signal
type
1
or
2
are
recognized
by
Pex
5
p
or
Pex
7
p
in
the
cytoplasm
and
are
imported
to
peroxisomes
via
translocation
machinery
.
In
regard
to
peroxisome-cytoplasmic
shuttling
of
Pex
5
p
,
Pex
5
p
initially
targets
to
an
800
-
kDa
docking
complex
consisting
of
Pex
14
p
and
Pex
13
p
and
then
translocates
to
a
500
-
kDa
RING
translocation
complex
.
At
the
terminal
step
,
Pex
1
p
and
Pex
6
p
of
the
AAA
family
mediate
the
export
of
Pex
5
p
,
where
Cys-ubiquitination
of
Pex
5
p
is
essential
for
the
Pex
5
p
exit
.
Diseases
Validation
Diseases presenting
"then translocates"
symptom
zellweger syndrome
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