Article
Expression of Amyloidogenic Transthyretin Drives Hepatic Proteostasis Remodeling in an Induced Pluripotent Stem Cell Model of Systemic Amyloid Disease.
Stem cell reports - 11 Aug 2020
Giadone Richard M, Liberti Derek C, Matte Taylor M, Rosarda Jessica D, Torres-Arancivia Celia, Ghosh Sabrina, Diedrich Jolene K, Pankow Sandra, Skvir Nicholas, Jean J C, Yates John R, Wilson Andrew A, Connors Lawreen H, Kotton Darrell N, Wiseman R Luke, Murphy George J
Abstract excerpt
The systemic amyloidoses are diverse disorders in which misfolded proteins are secreted by effector organs and deposited as proteotoxic aggregates at downstream tissues. Although well described clinically, the contribution of synthesizing organs to amyloid disease pathogenesis is unknown. Here, we utilize hereditary transthyretin amyloidosis (ATTR amyloidosis) induced pluripotent stem cells (iPSCs) to define the...
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