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Dilated Cardiomyopathy and Creutzfeldt-Jakob Disease: Evidence for a Role of Cellular Prion Protein in the Heart?
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We read with great interest the case published by Ashwath et al.1 The authors describe a patient with new-onset dilated cardiomyopathy after Creutzfeldt-Jakob disease. An endomyocardial biopsy specimen showed abnormal prion protein (PrPSc) accumulation in muscle fibers and peripheral heart nerves. This is an important observation that might have consequences regarding the understanding of the physiological functions of cellular prion (PrPc) in the heart.
Although only PrPSc was observed by Ashwath et al,1 PrPc is expressed in cardiac and skeletal muscles,2 and its physiological functions have been largely discussed.3 PrPc has a protective role against both programmed cell death and oxidative stress.3 Also, we observed that PrPc gene-ablated mice (PrP0/0) presented impaired tolerance to physical activity4 and higher levels of oxidative stress markers in both skeletal and myocardial muscles.5 The heart is a muscle under constant stress, and free radicals as well as apoptotic mechanisms have been . . . [Full Text of this Article]
AUTHOR INFORMATION
Marino Muxfeldt Bianchin, MD, PhD;
Roger Walz, MD, PhD;
Ricardo R. Brentani, MD, PhD;
Vilma R. Martins, PhD
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