Abstract
Although more than 90% systemic mastocytosis (SM) patients express gain of function mutations in the KIT receptor, recent next generation sequencing has revealed the presence of several additional genetic and epigenetic mutations in a subset of these patients, which confer poor prognosis and inferior overall survival. A clear understanding of how genetic and epigenetic mutations cooperate in regulating the tremendous heterogeneity observed in these patients will be essential for designing effective treatment strategies for this complex disease. In this review, we describe the clinical heterogeneity observed in patients with mastocytosis, the nature of relatively novel mutations identified in these patients, therapeutic strategies to target molecules downstream from activating KIT receptor and finally we speculate on potential novel strategies to interfere with the function of not only the oncogenic KIT receptor but also epigenetic mutations seen in these patients.
| Original language | English |
|---|---|
| Pages (from-to) | 18250-18264 |
| Number of pages | 15 |
| Journal | Oncotarget |
| Volume | 6 |
| Issue number | 21 |
| State | Published - 2015 |
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Keywords
- Alternative targets in mastocytosis
- KIT mutations
- Mastocytosis
- Myeloproliferative disorder
- Signaling pathways in mastocytosis
ASJC Scopus subject areas
- Oncology
Cite this
Mastocytosis- a mutated KIT receptor induced myeloproliferative disorder. / Chatterjee, Anindya; Ghosh, Joydeep; Kapur, Reuben.
In: Oncotarget, Vol. 6, No. 21, 2015, p. 18250-18264.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Mastocytosis- a mutated KIT receptor induced myeloproliferative disorder
AU - Chatterjee, Anindya
AU - Ghosh, Joydeep
AU - Kapur, Reuben
PY - 2015
Y1 - 2015
N2 - Although more than 90% systemic mastocytosis (SM) patients express gain of function mutations in the KIT receptor, recent next generation sequencing has revealed the presence of several additional genetic and epigenetic mutations in a subset of these patients, which confer poor prognosis and inferior overall survival. A clear understanding of how genetic and epigenetic mutations cooperate in regulating the tremendous heterogeneity observed in these patients will be essential for designing effective treatment strategies for this complex disease. In this review, we describe the clinical heterogeneity observed in patients with mastocytosis, the nature of relatively novel mutations identified in these patients, therapeutic strategies to target molecules downstream from activating KIT receptor and finally we speculate on potential novel strategies to interfere with the function of not only the oncogenic KIT receptor but also epigenetic mutations seen in these patients.
AB - Although more than 90% systemic mastocytosis (SM) patients express gain of function mutations in the KIT receptor, recent next generation sequencing has revealed the presence of several additional genetic and epigenetic mutations in a subset of these patients, which confer poor prognosis and inferior overall survival. A clear understanding of how genetic and epigenetic mutations cooperate in regulating the tremendous heterogeneity observed in these patients will be essential for designing effective treatment strategies for this complex disease. In this review, we describe the clinical heterogeneity observed in patients with mastocytosis, the nature of relatively novel mutations identified in these patients, therapeutic strategies to target molecules downstream from activating KIT receptor and finally we speculate on potential novel strategies to interfere with the function of not only the oncogenic KIT receptor but also epigenetic mutations seen in these patients.
KW - Alternative targets in mastocytosis
KW - KIT mutations
KW - Mastocytosis
KW - Myeloproliferative disorder
KW - Signaling pathways in mastocytosis
UR - http://www.scopus.com/inward/record.url?scp=84938804012&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84938804012&partnerID=8YFLogxK
M3 - Article
C2 - 26158763
AN - SCOPUS:84938804012
VL - 6
SP - 18250
EP - 18264
JO - Oncotarget
JF - Oncotarget
SN - 1949-2553
IS - 21
ER -