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Biosynthesis and trafficking of the bile salt export pump, BSEP: therapeutic implications of BSEP mutations.
[benign recurrent intrahepatic cholestasis]
The
bile
salt
export
pump
(
BSEP
,
ABCB
11
)
is
the
primary
transporter
of
bile
acids
from
the
hepatocyte
to
the
biliary
system
.
This
rate-limiting
step
in
bile
formation
is
essential
to
the
formation
of
bile
salt
dependent
bile
flow
,
the
enterohepatic
circulation
of
bile
acids
,
and
the
digestion
of
dietary
fats
.
Mutations
in
BSEP
are
associated
with
cholestatic
diseases
such
as
progressive
familial
intrahepatic
cholestasis
type
2
(
PFIC
2
)
,
benign
recurrent
intrahepatic
cholestasis
type
2
(
BRIC
2
)
,
drug-induced
cholestasis
,
and
intrahepatic
cholestasis
of
pregnancy
.
Development
of
clinical
therapies
for
these
conditions
necessitates
a
clear
understanding
of
the
cell
biology
of
biosynthesis
,
trafficking
,
and
transcriptional
and
translational
regulation
of
BSEP
.
This
chapter
will
focus
on
the
molecular
and
cell
biological
aspects
of
this
critical
hepatic
membrane
transporter
.
