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Characterizing Myeloid Cell Activation in NF1 Vasculopathy

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Technical Report,01 Jul 2015,30 Jun 2016

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Georgia Regents University Augusta United States

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The overarching theme of our NF YI proposal is to gain mechanistic insight and develop therapeutic targets for the preventiontreatment of neurofibromatosis type 1 NF1 related cardiovascular disease. Previously, we developed mouse models of arterial stenosis and aneurysm formation that resemble disease in NF1 patients. We have completely interrogated the MCP1CCR2 signaling pathway in the pathogenesis of NF1 arterial stenosis and a published manuscript is attached to this report. The primary findings of these studies were that CCR2 activation is required for the infiltration of neurofibromin-deficient myeloid cells, the primary cellular effectors of NF1 arterial stenosis, and that pharmacologic inhibition of CCR2 activation is a viable therapeutic option for the prevention andor treatment of NF1 arterial stenosis. The second aim of our proposal was to interrogate reactive oxygen species production in the generation of NF1 aneurysms. We have initiated work on the proposed experiments using our NF1-related aneurysm model and have generated the mice and tissues for the proposed experiments. We have also begun experiments in a separate model system calcium chloride to support our previous findings of enhanced aneurysm formation in Nf1 heterozygous mice.

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  • Medicine and Medical Research

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