Loss or inhibition of lysosomal acid lipase <i>in vitro</i> leads to cholesteryl ester accumulation without affecting muscle formation or mitochondrial function.

ABSTRACT: Skeletal muscle (SM) is essential for movement, stability, and overall body function, and it readily adapts to changes in energy demand. Myogenesis is energy-intensive and involves complex molecular and cellular events. We recently demonstrated that the absence of lysosomal acid lipase (LAL) in vivo significantly impacts the SM phenotype, primarily by disrupting energy homeostasis and reducing ATP production. As systemic LAL deficiency affects multiple organs, we hypothesized that the altered SM phenotype resulted from systemic rather than SM-specific loss of LAL activity. To distinguish between systemic and cell-intrinsic effects, we used primary myoblasts isolated from Lal-deficient (-/-) mice as well as C2C12 cells treated with the pharmacological inhibitor of LAL, Lalistat-2. We

SUBMITTER: Akhmetshina A 

PROVIDER: S-EPMC11745973 | biostudies-literature | 2025

REPOSITORIES: biostudies-literature