Heart failure reversed in Marfan mice
Cell signals responsible for rapid heart failure in children with Marfan syndrome and reverse the disease in mouse models
- Date:
- November 14, 2017
- Source:
- Johns Hopkins Medicine
- Summary:
- In experiments with mice that have a rodent form of Marfan syndrome, researchers report that even modestly increasing stress on the animals' hearts -- at levels well-tolerated in normal mice -- can initiate heart failure. The findings revealed a novel cellular pathway in heart tissue that leads to heart failure and may serve as a model for a new standard of treatment for children with this aggressive form of Marfan syndrome.
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In experiments with mice that have a rodent form of Marfan syndrome, Johns Hopkins researchers report that even modestly increasing stress on the animals' hearts -- at levels well-tolerated in normal mice -- can initiate heart failure. The findings, described August 4 in the Journal of Clinical Investigation Insight, revealed a novel cellular pathway in heart tissue that leads to heart failure and may serve as a model for a new standard of treatment for children with this aggressive form of Marfan syndrome.
Marfan syndrome is a genetic disorder that affects connective tissue throughout the body, elongating limbs, fingers and toes, for example. However, its worst effects are in the heart's blood vessels and valves. Aortic enlargement, heart valve leaks and heart failure -- marked by heart enlargement and weakened pumping action -- are all potentially life-threatening.
The Hopkins team's interest in the mouse model grew out of the clinical experience of children with Marfan seen at The Johns Hopkins Hospital over decades.
Story Source:
Materials provided by Johns Hopkins Medicine. Note: Content may be edited for style and length.
Journal Reference:
- Rosanne Rouf, Elena Gallo MacFarlane, Eiki Takimoto, Rahul Chaudhary, Varun Nagpal, Peter P. Rainer, Julia G. Bindman, Elizabeth E. Gerber, Djahida Bedja, Christopher Schiefer, Karen L. Miller, Guangshuo Zhu, Loretha Myers, Nuria Amat-Alarcon, Dong I. Lee, Norimichi Koitabashi, Daniel P. Judge, David A. Kass, Harry C. Dietz. Nonmyocyte ERK1/2 signaling contributes to load-induced cardiomyopathy in Marfan mice. JCI Insight, 2017; 2 (15) DOI: 10.1172/jci.insight.91588
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