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(American Journal of Pathology. 2006;169:761-773.)
© 2006 American Society for Investigative Pathology
DOI: 10.2353/ajpath.2006.060250

BAG3 Deficiency Results in Fulminant Myopathy and Early Lethality

Sachiko Homma*, Masahiro Iwasaki{dagger}, G. Diane Shelton{ddagger}, Eva Engvall*, John C. Reed* and Shinichi Takayama*

From the Burnham Institute for Medical Research,* La Jolla, California; the Center for Molecular Chaperone Biology,{dagger} Cancer Research Center, Medical College of Georgia, Augusta, Georgia; and the Department of Pathology,{ddagger} University of California, San Diego, La Jolla, California

Bcl-2-associated athanogene 3 (BAG3) is a member of a conserved family of cyto-protective proteins that bind to and regulate Hsp70 family molecular chaperones. Here, we show that BAG3 is prominently expressed in striated muscle and colocalizes with Z-disks. Mice with homozygous disruption of the bag3 gene developed normally but deteriorated postnatally with stunted growth evident by 1 to 2 weeks of age and death by 4 weeks. BAG3-deficient animals developed a fulminant myopathy characterized by noninflammatory myofibrillar degeneration with apoptotic features. Knockdown of bag3 expression in cultured C2C12 myoblasts increased apoptosis on induction of differentiation, suggesting a need for bag3 for maintenance of myotube survival and confirming a cell autonomous role for bag3 in muscle. We conclude that although BAG3 is not required for muscle development, this co-chaperone appears to be critically important for maintenance of mature skeletal muscle.




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