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Aberrant reduction of MiR-141 increased CD47/CUL3 in Hirschsprung's disease.
[hirschsprung disease]
MiR-
141
has
been
confirmed
to
be
associated
with
various
human
diseases
.
However
,
whether
miR-
141
is
involved
in
the
pathogenesis
of
Hirschsprung
's
disease
(
HSCR
)
remains
unknown
.
Here
,
we
design
the
experiment
to
reveal
the
relationship
between
miR-
141
and
HSCR
.
Quantitative
real-time
PCR
and
Western
blot
were
used
to
detect
the
expression
levels
of
miR-
141
and
its
potential
genes
in
70
tissues
of
HSCR
compared
with
60
controls
.
Bisulfite
sequencing
PCR
(
BSP
)
assay
was
applied
to
explain
the
possible
mechanism
of
the
aberrant
expression
level
of
miR-
141
.
We
employed
a
dual-luciferase
reporter
assay
to
validate
the
regulation
relation
between
miR-
141
and
CD
47
/
CUL
3
.
Cell
migration
,
proliferation
,
apoptosis
,
and
cell
cycle
progression
were
examined
by
transwell
assay
,
MTT
assay
,
and
flow
cytometry
,
respectively
.
M
iR-
141
was
down-regulated
whereas
CD
47
and
CUL
3
expression
was
increased
in
colon
tissues
from
patients
with
HSCR
compared
with
control
group
,
The
increased
level
of
CD
47
and
CUL
3
induced
by
miR-
141
reduced
proliferation
and
migration
of
293
T
and
SH-
SY
5
Y
cells
.
Furthermore
,
this
suppression
was
reversed
by
reducing
of
CD
47
and
CUL
3
.
Hypermethylation
of
a
CpG
Island
in
the
promoter
region
of
miR-
141
gene
was
confirmed
in
HSCR
tissues
.
Aberrant
reduction
of
miR-
141
may
play
an
important
role
in
the
pathogenesis
of
HSCR
with
the
inhibiting
affection
on
cell
migration
and
proliferation
abilities
.
The
present
study
demonstrates
for
the
first
time
the
role
of
miR-
141
and
its
target
genes
in
the
occurrence
of
HSCR
,
and
provides
us
a
new
direction
for
the
study
of
the
pathogenesis
of
Hirschsprung
's
disease
.
Diseases
Validation
Diseases presenting
"first time"
symptom
achondroplasia
acute rheumatic fever
adrenal incidentaloma
adrenomyeloneuropathy
alpha-thalassemia
aniridia
aromatase deficiency
canavan disease
carcinoma of the gallbladder
cholangiocarcinoma
classical phenylketonuria
congenital adrenal hyperplasia
congenital toxoplasmosis
cowden syndrome
cushing syndrome
cutaneous mastocytosis
dedifferentiated liposarcoma
dentin dysplasia
dentinogenesis imperfecta
dracunculiasis
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
erythropoietic protoporphyria
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
fabry disease
familial mediterranean fever
gm1 gangliosidosis
harlequin ichthyosis
heparin-induced thrombocytopenia
hirschsprung disease
hodgkin lymphoma, classical
holt-oram syndrome
hydrocephalus with stenosis of the aqueduct of sylvius
junctional epidermolysis bullosa
kabuki syndrome
kallmann syndrome
liposarcoma
locked-in syndrome
lymphangioleiomyomatosis
malignant atrophic papulosis
megacystis-microcolon-intestinal hypoperistalsis syndrome
monosomy 21
neuralgic amyotrophy
oculocutaneous albinism
oligodontia
omenn syndrome
oral submucous fibrosis
papillon-lefèvre syndrome
pendred syndrome
phenylketonuria
primary effusion lymphoma
primary hyperoxaluria type 1
severe combined immunodeficiency
sneddon syndrome
triple a syndrome
trochlear dysplasia
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
werner syndrome
wiskott-aldrich syndrome
wolf-hirschhorn syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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