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Stem cell factor contributes to tumorigenesis of mast cells via an autocrine/paracrine mechanism.
[cutaneous mastocytosis]
Mastocytosis
is
a
disease
accompanied
by
the
abnormal
expansion
and
accumulation
of
mast
cells
.
Although
the
D
816
V
mutation
is
detected
in
most
cases
of
systemic
mastocytosis
,
the
mutation
is
rarely
observed
in
other
forms
of
mastocytosis
,
such
as
cutaneous
mastocytosis
and
mast
cell
leukemia
/
sarcoma
,
for
which
the
mechanism
of
tumorigenesis
remains
unknown
.
In
this
study
,
we
demonstrated
a
novel
mechanism
of
mast
cell
tumorigenesis
via
SCF
autocrine
/
paracrine
release
.
SCF
was
highly
expressed
in
a
WT
KIT
-expressing
HRMC
line
,
contributing
to
the
phosphorylation
of
KIT
.
Neutralization
of
external
SCF
using
a
neutralizing
antibody
or
suppression
of
SCF
production
by
RNA
interference
inhibited
the
growth
of
HRMC
cells
,
indicating
the
essential
role
of
SCF
in
cell
proliferation
.
To
the
best
of
our
knowledge
,
this
is
the
first
report
to
determine
the
significant
contribution
of
SCF
autoproduction
to
neoplastic
proliferation
of
mast
cells
.
These
results
indicate
the
possibility
that
targeting
SCF
production
may
become
a
novel
treatment
for
mast
cell
malignancies
.
Diseases
Validation
Diseases presenting
"first report"
symptom
achondroplasia
alexander disease
aniridia
cadasil
canavan disease
child syndrome
cohen syndrome
congenital toxoplasmosis
cowden syndrome
cushing syndrome
cutaneous mastocytosis
cystinuria
dedifferentiated liposarcoma
dentinogenesis imperfecta
dracunculiasis
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
esophageal squamous cell carcinoma
fabry disease
familial mediterranean fever
focal myositis
harlequin ichthyosis
hirschsprung disease
hodgkin lymphoma, classical
holt-oram syndrome
homocystinuria without methylmalonic aciduria
inclusion body myositis
junctional epidermolysis bullosa
kabuki syndrome
kindler syndrome
krabbe disease
lamellar ichthyosis
liposarcoma
lymphangioleiomyomatosis
monosomy 21
neonatal adrenoleukodystrophy
neuralgic amyotrophy
oculocutaneous albinism
oligodontia
omenn syndrome
pendred syndrome
pleomorphic liposarcoma
primary hyperoxaluria type 1
pyomyositis
pyruvate dehydrogenase deficiency
scrub typhus
severe combined immunodeficiency
sneddon syndrome
triple a syndrome
typhoid
waldenström macroglobulinemia
werner syndrome
wiskott-aldrich syndrome
x-linked adrenoleukodystrophy
zellweger syndrome
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