Scientists at the University of Cologne in Germany have identified a protein that blocks accumulation of the toxic protein aggregates that cause neurodegeneration in Huntington’s disease. Studies by research head David Vilchez, Ph.D.,and colleagues at the University of Cologne’s Cluster of Excellence for Aging Research (CECAD), found that a protein known as UBR5 promotes degradation of the mutant huntingtin protein (HTT) in induced pluripotent stem cells (IPSCs) derived from human Huntington’s disease (HD) patients. Their experiments in invertebrate models of the disease also showed that while lack of the worm’s equivalent protein promotes neurotoxicity, overexpression induced breakdown of mutant HTT.

The findings, reported in Nature Communications , could help scientists to better understand how Huntington’s disease develops, and potentially lead to new therapeutic approaches. The team’s published paper is titled, “T he ubiquitin ligase UBR5 suppresses proteostasis collapse in pluripotent stem cells from Huntington’s disease patients .” …