BAG3 Deficiency Results in Fulminant Myopathy and Early Lethality
Open accessAbstract
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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Loss-of-function of Bag3 causes severe myopathy symptoms in mice and humans.
“The importance of Bag3 for skeletal muscle is further demonstrated by the finding that a loss-of-function of Bag3 causes severe myopathy symptoms in mice and humans (62, 117).”
From The Hippo signal transduction network for exercise physiologists (2016) · cites this paper as [62]
The paper’s opening summary reports that mice with homozygous Bag3 disruption developed fulminant myopathy, and the Discussion describes severe muscle degeneration and death before four weeks. The passages about humans concern BAG3 expression, not loss-of-function causing myopathy in humans; this source supports the mouse portion of the claim but not the human portion. (LLM, conf=0.88)
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IMMUNE STATUS AND APOPTOSIS ACTIVATION DURING BRAIN DEATH
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- Authors
- Homma, S., Iwasaki, M., Shelton, G. D., Engvall, E., Reed, J. C., & Takayama, S.
- Affiliations
- Sanford Burnham Prebys Medical Discovery Institute (US)
- Augusta University (US)
- University of California San Diego (US)
- Venue
- The American Journal of Pathology
- Publisher
- Elsevier BV
- Published
- 2006-09
- Volume
- 169
- Issue
- 3
- Pages
- 761-773
- ISSN
- 0002-9440 (ISSN-L)
- PMC
- PMC1698816
- OpenAlex
- W2018956321
- Cited by
- 243
- References
- 46
- Citations by year
- 2026: 3 · 2025: 12 · 2024: 11 · 2023: 16 · 2022: 12 · 2021: 20 · 2020: 15 · 2019: 12 · 2018: 11 · 2017: 16 · 2016: 22 · 2015: 18 · 2014: 10 · 2013: 7 · 2012: 8
- FWCI
- 2.18
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- American Journal Of Pathology (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health (CA67329, HD31636, CA107793)
- Muscular Dystrophy Association
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Muscle Physiology and Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Heat shock proteins research
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiomyopathy and Myosin Studies
- Keywords
- BAG3, Hsp70 chaperones, co-chaperones, skeletal muscle, myopathy, Z-disc, myofibrillar degeneration, apoptosis, myogenesis, C2C12 myoblasts, knockout mice, postnatal lethality, muscle maintenance
- MeSH terms
- Animals, Carrier Proteins, Cell Differentiation, Cell Line, Cell Survival, Humans, Muscular Diseases, Protein Binding, Time Factors, Apoptosis, Mice, Knockout, Muscle, Skeletal, Muscle Fibers, Skeletal, HSP70 Heat-Shock Proteins, Muscle Development, Myoblasts, Skeletal, Adaptor Proteins, Signal Transducing, Apoptosis Regulatory Proteins, Mice
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