Published: 03 April, 2020 | Volume 3 - Issue 1 | Pages: 001-020
Figure 2:
The heterozygous JAK2V617F mutated acquired thrombocythemia and germline TPO and JAK2V671I or JAK2Q534R mutated hereditary thrombocythemia as well as acquired MPL515 and congenital hereditary MPL505 mutated thrombocythemia (ET) are driven by indirect cytokine activation heterozygous JAK2V617F→STAT5, germline JAK2 or TPO→TpoR = MPL. Direct binding of TPO to the D3D4 domain of TpoRor direct cytokine activation (MPL515 and MPL505) cytokine receptor activation induce an ET phenotype of MPN without features of PV (EEC negative) Michiels, et al. 2014 [27]. CALR mutants Type 1 and 2, but not wild type CALR, did induce STAT5 activation via TpoR (MPL) and GCSFR, but not via EpoR. The STAT5 activation via GSCFR was much weak erthan via TpoR (MPL). The extracelllar domain of TpoR (MPL), but not of EpoR, was indispensible for CALR mutant induced activity and the D1D2 distal part of the extracelluar TpoR domain and its associated N-glycosylation sites but not the TPO binding site in the D3D4 domain Araki, et al. Vainchenker and Kralovics 2017) [65].
Read Full Article HTML DOI: 10.29328/journal.ijbmr.1001011 Cite this Article Read Full Article PDF
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