title: Axonopathy in Huntington's disease.
abstract: Personality changes, psychiatric disturbances and cognitive abnormalities frequently characterise the prodromal phase in Huntington's disease (HD), a devastating monogenic neurodegenerative disorder manifesting with abnormal motor movements and early death. Selective loss of medium-sized spiny striatal neurons has been related to the onset of motor symptoms but it does not completely explain the psychiatric and cognitive changes that often precede motor abnormalities. Here we review the evidence of synaptic and axonal dysfunction and neurite dystrophy preceding neuronal loss in HD patients and models. We discuss possible mechanisms leading to dysfunction of the axonal and synaptic compartments and identify potential novel targets for effective therapeutic intervention.
authors.affiliation: Neural Plasticity and Repair Unit, Wallenberg Neuroscience Center, Lund University, BMC A10, 22184 Lund, Sweden. jia-yi.li@med.lu.se
authors.forename: Jia-Yi
authors.initials: JY
authors.lastname: Li
authors.forename: Laura
authors.initials: L
authors.lastname: Conforti
mesh_terms: D000818:Animals; D001369:Axons / Q000378:metabolism* / Q000473:pathology*; D006801:Humans; D000071058:Huntingtin Protein; D006816:Huntington Disease / Q000378:metabolism* / Q000473:pathology*; D009419:Nerve Tissue Proteins / Q000378:metabolism; D009474:Neurons / Q000378:metabolism / Q000473:pathology; D021381:Protein Transport / Q000502:physiology
chemical_list: C086055:HTT protein, human; D000071058:Huntingtin Protein; D009419:Nerve Tissue Proteins