title: New transgenic ALS/FTD models on the rat-walk: An Editorial Highlight for 'Increased Ubqln2 expression causes neuron death in transgenic rats'.
abstract: This Editorial highlights a study by Huang and colleagues in the current issue of Journal of Neurochemistry. The authors introduce a novel ALS-FTD (amyotrophic lateral sclerosis-frontotemporal dementia) rat model to explore the role of the UBLQN2 gene that has previously been associated with familial ALS-FTD. Over-expression of ubiquilin 2 in the cortex (CTX) and hippocampus of the rat results in ubiquilin 2 aggregates and neurodegeneration together with cognitive deficits. The new rat model not only gives insight into potential molecular underpinnings of ALS-FTD, but also represents an important new tool for future research and therapeutic approaches. Read the highlighted article 'Increased Ubqln2 expression causes neuron death in transgenic rats' on page 285.
authors.affiliation: Department of Neurology, University Clinics, RWTH Aachen, Aachen, Germany. jstegmueller@ukaachen.de.
authors.forename: Judith
authors.initials: J
authors.lastname: Stegmüller
authors.affiliation: Department of Neurodegenerative Diseases, Hertie Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany.|German Research Center for Neurodegenerative Diseases (DZNE), University of Tübingen, Tübingen, Germany.
authors.forename: Matthis
authors.initials: M
authors.lastname: Synofzik
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics* / Q000473:pathology; D000818:Animals; D001343:Autophagy; D016923:Cell Death; D057174:Frontotemporal Lobar Degeneration / Q000235:genetics* / Q000473:pathology; D009474:Neurons / Q000473:pathology*; D051381:Rats; D055647:Rats, Transgenic / Q000235:genetics*; D014452:Ubiquitins / Q000235:genetics*
chemical_list: D014452:Ubiquitins; C000603109:Ubqln2 protein, rat