"Humans"

Mesencephalic astrocyte-derived neurotrophic factor is an ER-resident chaperone that protects against reductive stress in the heart.

We have previously demonstrated that ischemia/reperfusion (I/R) impairs endoplasmic reticulum (ER)-based protein folding in the heart and thereby activates an unfolded protein response sensor and effector, activated transcription factor 6α (ATF6). …

Designing Novel Therapies to Mend Broken Hearts: ATF6 and Cardiac Proteostasis.

The heart exhibits incredible plasticity in response to both environmental and genetic alterations that affect workload. Over the course of development, or in response to physiological or pathological stimuli, the heart responds to fluctuations in …

Proteostasis and Beyond: ATF6 in Ischemic Disease.

Endoplasmic reticulum (ER) stress is a pathological hallmark of numerous ischemic diseases, including stroke and myocardial infarction (MI). In these diseases, ER stress leads to activation of the unfolded protein response (UPR) and subsequent …

Unfolding the Roles of Mitochondria as Therapeutic Targets for Heart Disease.

Pharmacologic ATF6 activation confers global protection in widespread disease models by reprograming cellular proteostasis.

Pharmacologic activation of stress-responsive signaling pathways provides a promising approach for ameliorating imbalances in proteostasis associated with diverse diseases. However, this approach has not been employed in vivo. Here we show, using a …

Pharmacologic ATF6 activating compounds are metabolically activated to selectively modify endoplasmic reticulum proteins.

Pharmacologic arm-selective unfolded protein response (UPR) signaling pathway activation is emerging as a promising strategy to ameliorate imbalances in endoplasmic reticulum (ER) proteostasis implicated in diverse diseases. The small molecule …

ER Protein Quality Control and the Unfolded Protein Response in the Heart.

Cardiac myocytes are the cells responsible for the robust ability of the heart to pump blood throughout the circulatory system. Cardiac myocytes grow in response to a variety of physiological and pathological conditions; this growth challenges …

ATF6 Decreases Myocardial Ischemia/Reperfusion Damage and Links ER Stress and Oxidative Stress Signaling Pathways in the Heart.

RATIONALE: Endoplasmic reticulum (ER) stress causes the accumulation of misfolded proteins in the ER, activating the transcription factor, ATF6 (activating transcription factor 6 alpha), which induces ER stress response genes. Myocardial ischemia …

Expanding the Paracrine Hypothesis of Stem Cell-Mediated Repair in the Heart: When the Unconventional Becomes Conventional.

Recent interest in mechanisms of stem cell-mediated repair in the heart have spawned the ``paracrine hypothesis'', which posits that stem cells release beneficial substances that improve regeneration and function of the injured and diseased …

Finding the missing link between the unfolded protein response and O-GlcNAcylation in the heart.

The ER stress-inducible transcription factor, x-box binding protein 1 (XBP1), which enhances protein glycosylation in the endoplasmic reticulum (ER), was shown to also enhance protein glycosylation outside the ER, via a process called …