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Bone marrow-derived cells promote liver regeneration in mice with erythropoietic protoporphyria.
[erythropoietic protoporphyria]
Bone
marrow
transplantation
can
reverse
hepatic
protoporphyrin
accumulation
and
prevent
the
hepatobiliary
complications
characteristic
of
erythropoietic
protoporphyria
.
The
aim
of
this
study
was
to
assess
the
recruitment
capacity
of
bone
marrow
cells
in
the
damaged
liver
and
their
possible
contribution
to
the
improved
or
recovered
hepatic
function
in
a
murine
model
of
erythropoietic
protoporphyria
(
EPP
)
.
Lethally
irradiated
female
EPP
mice
were
transplanted
with
bone
marrow
cells
from
healthy
male
mice
and
were
monitored
during
12
or
36
weeks
.
Two
groups
of
animals
killed
12
weeks
after
transplant
were
also
treated
with
granulocyte
colony-stimulating
factor
.
C
ell
transplantation
decreased
porphyrin
contents
in
erythrocytes
and
liver
.
Improved
hepatic
structure
and
function
and
reduced
hepatic
fibrosis
were
observed
,
especially
36
weeks
after
transplant
.
Bone
marrow-derived
cells
(
22
%
-
35
%
)
were
identified
in
the
liver
of
recipient
mice
by
means
of
fluorescence
in
situ
hybridization
(
chrY-FISH
)
or
green
fluorescent
protein
staining
and
were
characterized
by
immunofluorescence
staining
.
The
livers
of
recipients
contained
20
%
to
30
%
myofibroblasts
(
alpha-smooth
muscle
actin-
positive
cells
)
,
40
%
CK
19
-
positive
cells
,
and
10
%
to
28
%
hepatocytes
(
albumin
-
positive
cells
)
derived
from
the
donor
bone
marrow
.
Bone
marrow-derived
cells
play
a
significant
role
in
restoring
and
regenerating
hepatic
tissue
in
EPP
mice
.
Hepatic
repair
was
associated
with
fibrogenesis
,
enhanced
by
granulocyte
colony-stimulating
factor
treatment
,
and
almost
normal
liver
structure
and
function
was
observed
in
the
long
term
(
36
weeks
posttransplant
)
.
