[HTML][HTML] BAG3 plays a central role in proteostasis in the heart

W Mizushima, J Sadoshima - The Journal of clinical …, 2017 - Am Soc Clin Investig
W Mizushima, J Sadoshima
The Journal of clinical investigation, 2017Am Soc Clin Investig
Proteinopathies are characterized by the accumulation of misfolded proteins, which
ultimately interfere with normal cell function. While neurological diseases, such as
Huntington disease and Alzheimer disease, are well-characterized proteinopathies, cardiac
diseases have recently been associated with alterations in proteostasis. In this issue of the
JCI, Fang and colleagues demonstrate that mice with cardiac-specific deficiency of the co-
chaperone protein BCL2-associated athanogene 3 (BAG3) develop dilated cardiomyopathy …
Proteinopathies are characterized by the accumulation of misfolded proteins, which ultimately interfere with normal cell function. While neurological diseases, such as Huntington disease and Alzheimer disease, are well-characterized proteinopathies, cardiac diseases have recently been associated with alterations in proteostasis. In this issue of the JCI, Fang and colleagues demonstrate that mice with cardiac-specific deficiency of the co-chaperone protein BCL2-associated athanogene 3 (BAG3) develop dilated cardiomyopathy that is associated with a destabilization of small HSPs as the result of a disrupted interaction between BAG3 and HSP70. Together, the results of this study suggest that strategies to upregulate BAG3 during cardiac dysfunction may be beneficial.
The Journal of Clinical Investigation