PGC-1α pathway dysregulation disrupts myofiber specification in a mouse model of SBMA
Curtis J. Kuo, Laura B. Chopp, Zhigang Yu, Luhan Ni, Hien T. Zhao, Janghoo Lim, Andrew P. Lieberman · 2026
Read PGC-1α pathway dysregulation disrupts myofiber specification in a mouse model of SBMA by Curtis J. Kuo, Laura B. Chopp, Zhigang Yu, Luhan Ni, Hien T. Zhao, Janghoo Lim, Andrew P. Lieberman (2026) in the Tower of Babel, a free online library of public-domain works preserved in clean, searchable text. No sign-up. The full text is served as a plain-text file at a predictable URL, where text is present: https://antarctic-labs.com/tower-of-babel/pmc-13461168.txt.
Description
Seven researchers trace how a muscle energy regulator goes wrong in a mouse model of spinal and bulbar muscular atrophy. That disease is genetic, caused by an expanded repeat in the androgen receptor gene. Muscle damage is a major part of the disease but poorly understood. Using a gene-targeted mouse, the team read gene activity nucleus by nucleus in skeletal muscle.
They found a disease-specific set of muscle nuclei replacing the normal fiber types. The switch tracked with failure of the pathway led by PGC-1α, a master regulator of fiber identity and metabolism, and the failure deepened with age, hormones, and repeat length. A gene-silencing drug aimed at the receptor partly restored the pathway. Combined gene and binding data suggest the expanded protein traps the regulator's partners, rewriting which genes stay on. The authors propose this as a shared mechanism across related repeat diseases.
Catalog record
| CREATOR | Curtis J. Kuo, Laura B. Chopp, Zhigang Yu, Luhan Ni, Hien T. Zhao, Janghoo Lim, Andrew P. Lieberman |
|---|---|
| YEAR | 2026 |
| COLLECTION | PAPERS |
| FORMAT | TXT |
| FILE SIZE | 48 KB |
| VERSION | 1.0 |
| LICENSE | CC BY 4.0 |
| RIGHTS | CC BY |
| SOURCE | Europe PMC |