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Protoporphyrin retention in hepatocytes and Kupffer cells prevents sclerosing cholangitis in erythropoietic protoporphyria mouse model.
[erythropoietic protoporphyria]
Chronic
,
progressive
hepatobiliary
disease
is
the
most
severe
complication
of
erythropoietic
protoporphyria
(
EPP
)
and
can
require
liver
transplantation
,
although
the
mechanisms
that
lead
to
liver
failure
are
unknown
.
We
characterized
protoporphyrin-
IX
(
PPIX
)
-
linked
hepatobiliary
disease
in
BALB
/
c
and
C
5
7
BL
/
6
(
Fechm
1
P
as
)
mice
with
mutations
in
ferrochelatase
as
models
for
EPP
.
Fechm
1
P
as
and
wild-
type
(
control
)
mice
were
studied
at
12
-
14
weeks
of
age
.
PPIX
was
quantified
;
its
distribution
in
the
liver
,
serum
levels
of
lipoprotein-
X
,
liver
histology
,
contents
of
bile
salt
and
cholesterol
phospholipids
,
and
expression
of
genes
were
compared
in
mice
of
the
BALB
/
c
and
C
5
7
BL
/
6
backgrounds
.
The
in
vitro
binding
affinity
of
PPIX
for
bile
components
was
determined
.
Compared
with
mice
of
the
C
5
7
BL
/
6
background
,
BALB
/
c
Fechm
1
Pas
mice
had
a
more
severe
pattern
of
cholestasis
,
fibrosis
with
portoportal
bridging
,
bile
acid
regurgitation
,
sclerosing
cholangitis
,
and
hepatolithiasis
.
In
C
5
7
BL
/
6
Fechm
1
Pas
mice
,
PPIX
was
sequestrated
mainly
in
the
cytosol
of
hepatocytes
and
Kupffer
cells
,
whereas
,
in
BALB
/
c
Fechm
1
Pas
mice
,
PPIX
was
localized
within
enlarged
bile
canaliculi
.
Livers
of
C
5
7
BL
/
6
Fechm
1
Pas
mice
were
protected
through
a
combination
of
lower
efflux
of
PPIX
and
reduced
synthesis
and
export
of
bile
acid
.
PPIX
binds
to
bile
components
and
disrupts
the
physiologic
equilibrium
of
phospholipids
,
bile
acids
,
and
cholesterol
in
bile
.
This
process
might
be
involved
in
pathogenesis
of
sclerosing
cholangitis
from
EPP
;
a
better
understanding
might
improve
diagnosis
and
development
of
reagents
to
treat
or
prevent
liver
failure
in
patients
with
EPP
.
Diseases
Validation
Diseases presenting
"although the mechanisms that lead to liver failure are unknown"
symptom
erythropoietic protoporphyria
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