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[Bile formation and cholestasis].
[benign recurrent intrahepatic cholestasis]
Transport
proteins
in
hepatocytes
and
bile
duct
epithelium
mediate
uptake
and
secretion
of
cholephilic
compounds
in
the
liver
and
are
involved
in
bile
formation
.
Many
of
these
proteins
have
recently
been
cloned
and
characterized
and
appear
to
belong
to
large
gene
families
.
Apart
from
the
liver
these
proteins
are
expressed
in
the
blood
-
brain
barrier
,
placenta
,
kidneys
,
lungs
,
intestine
and
seminiferous
tubules
.
Prokaryotes
and
yeasts
contain
similar
proteins
.
In
cancer
cells
they
are
involved
in
multidrug
resistance
.
Some
genetic
cholestatic
liver
diseases
,
including
progressive
familial
intrahepatic
cholestasis
,
Dubin-
Johnson
syndrome
,
benign
recurrent
intrahepatic
cholestasis
and
intrahepatic
cholestasis
of
pregnancy
result
from
mutations
in
transport
protein
genes
.
These
proteins
also
play
a
role
in
drug-induced
liver
disease
and
in
primary
biliary
cirrhosis
.
Cyclosporine
and
oestradiol
(
glucuronide
)
for
instance
inhibit
bile
salt
export
protein
(
BSEP
)
.
Diseases
Validation
Diseases presenting
"placenta"
symptom
benign recurrent intrahepatic cholestasis
congenital diaphragmatic hernia
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