Journal of Cell Biology volume 215, issue 4, P531-542 2016 DOI: 10.1083/jcb.201607099 View full text
|
|
Share
Xinghua Yin, Grahame J. Kidd, Nobuhiko Ohno, Guy A. Perkins, Mark H. Ellisman, Chinthasagar Bastian, Sylvain Brunet, Selva Baltan, Bruce D. Trapp

Abstract: The authors show that central nervous system myelin lacking proteolipid protein (PLP) induces mitochondrial dysfunction, including altered motility, degeneration, and ectopic smooth endoplasmic reticulum interactions, leading to axonal structural defects and degeneration. Mutated PLP occurs in hereditary spastic paraplegia, and these cellular effects provide potential insight into the pathology of the disease.