Juvenile Myelomonocytic Leukemia
|
0.400 |
GeneticVariation
|
disease |
BEFREE |
We screened RNA from six patients with JCML for mutations in the GM-CSF receptor (GM-CSFR) coding sequence using RT-PCR-SSCP.
|
9131005 |
1997 |
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 |
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 |
GeneticVariation
|
disease |
BEFREE |
Our findings identify the molecular basis for the autocrine TNFalpha activation of the GM-CSF gene in JMML and suggest potential novel and specific approaches for the treatment of this aggressive childhood leukemia.
|
9185524 |
1997 |
Juvenile Myelomonocytic Leukemia
|
0.400 |
Biomarker
|
disease |
BEFREE |
JCML is characterized by a spontaneous growth of granulocyte-macrophage progenitors that show a striking hypersensitivity to granulocyte-macrophage colony-stimulating factor.
|
7672722 |
1995 |
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 |
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 |
Despite the presence of a measurable amount of GM-CSF in JCML cell culture supernatants, GM-CSF-specific mRNA in CFU-C cells of four cases was not detected by RT-PCR.
|
8286744 |
1994 |
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 |
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 |
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 |
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 |
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 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 |
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 |
Juvenile myelomonocytic leukemia (JMML) is characterized by hypersensitivity to granulocyte-macrophage colony-stimulating factor (GM-CSF).
|
23696637 |
2013 |
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 |
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 |
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 |
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 |
The selective sensitivity to DT388-GM-CSF of leukemic progenitors from a majority of JMML and CMML patients suggests that this agent could have therapeutic potential for some patients with these diseases.
|
9834234 |
1998 |
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 |
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 |
Leukemia cells from patients with juvenile myelomonocytic leukemia display hypersensitivity to certain cytokines, such as granulocyte-macrophage colony-stimulating factor.
|
27418650 |
2016 |