Effects of Sunitinib and Other Kinase Inhibitors on Cells Harboring a PDGFRB Mutation Associated with Infantile Myofibromatosis
نویسندگان
چکیده
منابع مشابه
Myeloproliferative Neoplasms Associated with Mutation in JAK2V617F and Tyrosine Kinase Inhibitors as Therapeutic Strategy
MPNs including a heterogeneous group of clonal or oligoclonal hamtopathies characterized by proliferation and accumulation of mature myeloid cells. JAK2 tyrosine kinase mutation is the most common molecular lesion identified in 90% of cases. JAK2 is involved in EPO signaling pathway, and mutations in it lead to EPO-independent spontaneous phosphorylation. Most tyrosine kinase inhibitors (TKI) a...
متن کاملModulation of expressivity in PDGFRB-related infantile myofibromatosis: a role for PTPRG?
Infantile myofibromatosis is a rare genetic disorder characterized by the development of benign tumors in the skin, muscle, bone, and viscera. The molecular pathogenesis is still incompletely known. An autosomal dominant form had been reported as causally related with mutations in the gene for platelet-derived growth factor receptor beta (PDGFRB). We report here two siblings with infantile myof...
متن کاملInfantile myofibromatosis*
Infantile myofibromatosis is a mesenchymal disorder characterized by the fibrous proliferation of the skin, bone, muscle and viscera. It is the most common fibrous tumor in childhood. We present a newborn with skin and bone disease without visceral involvement, who showed good response to vinblastine and methotrexate. Clinical features, etiology, diagnosis, and treatment are reviewed.
متن کاملInfantile Myofibromatosis
Infantile myofibromatosis (IMP) is a rare tumour with a wide spectrum of disease activity ra a solitarY cutaneous nodule through to a multicentric form ,vith widespread visceral involveluent. R is charactelised by its unique ability to spontaneously regress and has a typical histological appearance of a~tin-positivefibroblasts arranged in whorls or fascicles and vessels in a pericytomatous patt...
متن کاملInfantile myofibromatosis.
Infantile myofibromatosis (IMF) is a rare tumour with a wide spectrum of disease activity ranging from a solitary cutaneous nodule through to a multicentric form with widespread visceral involvement. It is characterised by its unique ability to spontaneously regress and has a typical histological appearance of actin-positive fibroblasts arranged in whorls or fascicles and vessels in a pericytom...
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ژورنال
عنوان ژورنال: International Journal of Molecular Sciences
سال: 2018
ISSN: 1422-0067
DOI: 10.3390/ijms19092599