Endoplasmic reticulum quality control (ERQC) pathways comprising chaperones, folding
enzymes, and degradation factors ensure the fidelity of ER protein folding and trafficking to …

Genetic, environmental, and aging-related insults can promote the misfolding and
subsequent aggregation of secreted proteins implicated in the pathogenesis of numerous …

Centenarians provide a unique lens through which to study longevity, healthy aging, and
resiliency. Moreover, models of human aging and resilience to disease that allow for the …

Serving as the metabolic hub of the human body, the liver is a vital organ that performs a
variety of important physiological functions. Although known for its regenerative potential, it …

The extracellular aggregation of destabilized transthyretin (TTR) variants is implicated in the
onset and pathogenesis of familial TTR-related amyloid diseases. One strategy to reduce …

Mammalian blood cells originate from specialized 'hemogenic'endothelial (HE) cells in
major arteries. During the endothelial-to-hematopoietic transition (EHT), nascent …

Hereditary transthyretin (TTR) amyloidosis (ATTRv amyloidosis) is autosomal dominant and
caused by mutation of TTR gene. Heterozygous ATTR Tyr114Cys (p. Tyr134Cys) …

Background In ATTR amyloidosis, transthyretin (TTR) protein is secreted from the liver and
deposited as toxic aggregates at downstream target tissues. Despite recent advancements …

Abstract Purpose of Review The advent of induced pluripotent stem cells (iPSC) has paved
the way for new in vitro models of human cardiomyopathy. Herein, we will review existing …

Transthyretin amyloidosis (ATTR) is characterized by the deposition of unstable transthyretin
protein (TTR) in the heart or peripheral nerves. Therapeutic strategies for ATTR include …