In a paper published online in advance in Nature this week, researchers at the Northwestern University Feinberg School of Medicine and elsewhere report that mutations in UBQLN2 — which encodes the ubiquitin-like protein ubiquilin 2 — "cause dominantly inherited, chromosome-X-linked ALS [amyotrophic lateral sclerosis] and ALS/dementia." In its functional analyses, the team found evidence linking "abnormalities in ubiquilin 2 to defects in the protein degradation pathway, abnormal