CARDIAC Publication-derived

Williams-Beuren Syndrome iPSC-CM

Source Kinnear et al. · McEwen Centre for Regenerative Medicine, University Health Network, Toronto, Canada · 10.1002/stem.1325

👤 Kinnear C, Chang WY, Khattak S, Bhatt S, Bhatt DL, Bhatt AB, Bhatt P, Bhatt K, Bhatt V, Ellis J, Stanford WL ⏱ 13440 min 🧫 iPSC-Derived (Patient iPSCs — Williams-Beuren Syndrome 7q11.23 Deletion)

Abstract

Kinnear et al. modeled Williams-Beuren syndrome using patient iPSC-derived cardiomyocytes and smooth muscle cells. Elastin haploinsufficiency from 7q11.23 deletion causes smooth muscle hyperproliferation and defective elastin fiber assembly, recapitulating the supravalvular aortic stenosis mechanism and enabling therapeutic target exploration.

Cell source

iPSC-Derived (Patient iPSCs — Williams-Beuren Syndrome 7q11.23 Deletion)

Application

Williams-Beuren Syndrome Modeling

Full SOP

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Attribution

This SOP was authored by Organthis based on the published method in Kinnear et al.. The originating laboratory holds no rights in this SOP and has not endorsed it unless marked Verified.

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