|کد مقاله||کد نشریه||سال انتشار||مقاله انگلیسی||ترجمه فارسی||نسخه تمام متن|
|2093219||1401336||2016||14 صفحه PDF||سفارش دهید||دانلود رایگان|
• Myeloid differentiation of murine iPSCs into mature Mφ (iPSC-Mφ)
• Functional iPSC-Mφ similar to bone marrow lineage-negative cell-derived Mφ
• Disease modeling of defects in GM-CSF signaling and Mφ function present in herPAP
SummaryInduced pluripotent stem cells (iPSCs) represent an innovative source for the standardized in vitro generation of macrophages (Mφ). We here describe a robust and efficient protocol to obtain mature and functional Mφ from healthy as well as disease-specific murine iPSCs. With regard to morphology, surface phenotype, and function, our iPSC-derived Mφ (iPSC-Mφ) closely resemble their counterparts generated in vitro from bone marrow cells. Moreover, when we investigated the feasibility of our differentiation system to serve as a model for rare congenital diseases associated with Mφ malfunction, we were able to faithfully recapitulate the pathognomonic defects in GM-CSF signaling and Mφ function present in hereditary pulmonary alveolar proteinosis (herPAP). Thus, our studies may help to overcome the limitations placed on research into certain rare disease entities by the lack of an adequate supply of disease-specific primary cells, and may aid the development of novel therapeutic approaches for herPAP patients.
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Journal: - Volume 7, Issue 2, 9 August 2016, Pages 292–305