|
Juvenile Myelomonocytic Leukemia
|
0.400 |
Biomarker
|
disease |
BEFREE |
Chronic myelomonocytic leukemia (CMML) and juvenile myelomonocytic leukemia (JMML) are myelodysplastic syndrome (MDS)/myeloproliferative neoplasm (MPN) overlap disorders characterized by monocytosis, myelodysplasia, and a characteristic hypersensitivity to granulocyte-macrophage colony-stimulating factor (GM-CSF).
|
28576879 |
2017 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
Patients with JMML have mutually exclusive genetic abnormalities in granulocyte-macrophage colony-stimulating factor (GM-CSF) receptor (GMR, CD116) signaling pathway.
|
26983639 |
2016 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
Biomarker
|
disease |
BEFREE |
Juvenile myelomonocytic leukemia (JMML) is an aggressive leukemia of early childhood characterized by aberrant proliferation of myelomonocytic cells and hypersensitivity to GM-CSF stimulation.
|
27447965 |
2016 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
Biomarker
|
disease |
BEFREE |
Leukemia cells from patients with juvenile myelomonocytic leukemia display hypersensitivity to certain cytokines, such as granulocyte-macrophage colony-stimulating factor.
|
27418650 |
2016 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
Biomarker
|
disease |
BEFREE |
Juvenile myelomonocytic leukaemia (JMML) is an aggressive myeloproliferative neoplasm in children characterized by granulocyte macrophage colony-stimulating factor (GM-CSF) hypersensitivity and resistance to chemotherapy.
|
24469048 |
2015 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
Biomarker
|
disease |
BEFREE |
Granulocyte-macrophage colony-stimulating factor (GM-CSF) also augmented the proliferation of JMML CD34(+) cells on AGM-S3 cells.
|
25102944 |
2015 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
Biomarker
|
disease |
BEFREE |
Following estimation of hypersensitivity to GM-CSF and genetic analysis of PTPN11, he was diagnosed with JMML in the blast crisis phase.
|
25047104 |
2014 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
Biomarker
|
disease |
BEFREE |
Notably, dasatinib, an U.S. Food and Drug Administration-approved multikinase inhibitor that also targets Src family, dramatically attenuated the spontaneous and GM-CSF-induced hypersensitive growth phenotype of mononuclear cells from peripheral blood and bone marrow collected from JMML patients harboring Cbl or other known JMML-associated mutations.
|
23400592 |
2013 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
Biomarker
|
disease |
BEFREE |
Juvenile myelomonocytic leukemia (JMML) is characterized by hypersensitivity to granulocyte-macrophage colony-stimulating factor (GM-CSF).
|
23696637 |
2013 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
In vitro differentiation of JMML iPSCs produced myeloid cells with increased proliferative capacity, constitutive activation of granulocyte macrophage colony-stimulating factor (GM-CSF), and enhanced STAT5/ERK phosphorylation, similar to primary JMML cells from patients.
|
23620576 |
2013 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
Biomarker
|
disease |
CTD_human |
Juvenile myelomonocytic leukemia is a lethal disease of children characterized by hypersensitivity of hematopoietic progenitors to granulocyte macrophage-colony stimulating factor.
|
22315502 |
2012 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
Biomarker
|
disease |
BEFREE |
It is characterized by hypersensitivity of JMML cells to granulocyte-macrophage colony-stimulating factor (GM-CSF) in vitro.
|
22480363 |
2012 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
Biomarker
|
disease |
BEFREE |
Juvenile myelomonocytic leukemia is a lethal disease of children characterized by hypersensitivity of hematopoietic progenitors to granulocyte macrophage-colony stimulating factor.
|
22315502 |
2012 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
Biomarker
|
disease |
BEFREE |
The biological hallmark of juvenile myelomonocytic leukemia (JMML) is selective GM-CSF hypersensitivity.
|
19010541 |
2009 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
Biomarker
|
disease |
BEFREE |
Mutations in RAS, neurofibromatosis type 1 (NF1), and PTPN11, constituents of the granulocyte-macrophage colony-stimulating factor signaling pathway, have been recognized in patients with juvenile myelomonocytic leukemia (JMML).
|
19047918 |
2009 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
Biomarker
|
disease |
BEFREE |
Specific defects in the RAS signalling pathway, which make JMML cells hypersensitive to granulocyte-macrophage colony-stimulating factor, are observed in at least two-thirds of patients with JMML: inactivation of NF1 or mutations in NRAS, KRAS2 or PTPN11.
|
18422786 |
2008 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
With this quantification method, JMML patients with RAS mutations showed significantly higher GM-CSF sensitivity than JMML patients with PTPN11 mutations.
|
18164384 |
2008 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
Biomarker
|
disease |
BEFREE |
These data indicate that SHIP-1 can effectively block GM-CSF hypersensitivity in JMML progenitor cells with mutations in KRAS2 or PTPN11 and may be a useful approach for the treatment of JMML patients.
|
17268534 |
2007 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
Mutations in SHP-2 phosphatase that cause hyperactivation of its catalytic activity have been identified in human leukemias, particularly juvenile myelomonocytic leukemia, which is characterized by hypersensitivity of myeloid progenitor cells to granulocyte macrophage colony-stimulating factor and interleukin (IL)-3.
|
16371368 |
2006 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
Biomarker
|
disease |
BEFREE |
These data support the hypothesis that PTPN11 mutations induce hematopoietic progenitor hypersensitivity to GM-CSF due to hyperactivation of the Ras signaling axis and provide a basis for the GM-CSF signaling pathway as a target for rational drug design in JMML.
|
15644411 |
2005 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
Hypersensitivity for granulocyte-macrophage colony-stimulating factor and pathologic activation of the Ras/MAPK pathway play an important role in the pathophysiology of juvenile myelomonocytic leukemia and provide the opportunity for several novel therapeutic approaches.
|
14521811 |
2003 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
Biomarker
|
disease |
BEFREE |
Granulocyte-macrophage colony-stimulating factor (GM-CSF) and tumor necrosis factor-alpha (TNF-alpha) promote JMML cell growth.
|
12010819 |
2002 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
Biomarker
|
disease |
BEFREE |
Chronic myelomonocytic leukemia requires granulocyte-macrophage colony-stimulating factor for growth in vitro and in vivo.
|
12384142 |
2002 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
Biomarker
|
disease |
BEFREE |
Human JMML and murine Nf1-deficient cells are hypersensitive to granulocyte-macrophage colony-stimulating factor (GM-CSF) in methylcellulose cultures.
|
10678181 |
2000 |
|
Juvenile Myelomonocytic Leukemia
|
0.400 |
AlteredExpression
|
disease |
BEFREE |
The stimulatory effect of TNF on GM-CSF gene expression in JMML cells probably takes place at the transcription level, because the ribozyme treatment decreased GM-CSF mRNA.
|
9834232 |
1998 |