ALS-associated protein plays a larger role in the aging process.
The Journal of Biological Chemistry has chosen a new study by Packard scientist Jiou Wang and colleagues as a “Paper of the Week.” JBC editor-in-chief said that Wang’s paper was in the top one percent of papers the journal reviewed every year in both significance and overall importance. Wang, a neuroscientist at Johns Hopkins University, showed that the worm version of the human protein TDP-43 is involved in the aging process and neurodegeneration by helping to regulate the turnover of other proteins in the cell.
Researchers had previously identified mutant forms of TDP-43 in some familial ALS patients, but they were unsure whether the mutant form of the protein actively harmed the cell or whether it was unable to perform its usual functions. To figure out exactly how TDP-43 might contribute to ALS, Wang and colleagues studied the worm Caenorhabditis elegans that lacked the nematode version of human TDP-43, known as TDP-1. C. elegans without TDP-1 had a greater tolerance to toxic proteins and consequently had a longer lifespan. TDP-43 mutations in humans, then, may contribute to ALS by creating problems with protein processing.