title: Embryonic Mutant Huntingtin Aggregate Formation in Mouse Models of Huntington's Disease.
abstract: The role of aggregate formation in the pathophysiology of Huntington's disease (HD) remains uncertain. However, the temporal appearance of aggregates tends to correlate with the onset of symptoms and the numbers of neuropil aggregates correlate with the progression of clinical disease. Using highly sensitive immunohistochemical methods we have detected the appearance of diffuse aggregates during embryonic development in the R6/2 and YAC128 mouse models of HD. These are initially seen in developing axonal tracts and appear to spread throughout the cerebrum in the early neonate.
authors.affiliation: Department of Biochemistry and Cellular and Molecular Biology, University of Tennessee, Knoxville, TN, USA.
authors.forename: Alexander P
authors.initials: AP
authors.lastname: Osmand
authors.affiliation: Department of Pediatrics, Vanderbilt University Medical Center, Nashville, TN, USA.
authors.forename: Terry Jo
authors.initials: TJ
authors.lastname: Bichell
authors.affiliation: Department of Pediatrics, Neurology, and Biochemistry, Vanderbilt University (VU) and VU Medical Center, Nashville, TN, USA.
authors.forename: Aaron B
authors.initials: AB
authors.lastname: Bowman
authors.affiliation: Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, London, UK.
authors.forename: Gillian P
authors.initials: GP
authors.lastname: Bates
mesh_terms: D000818:Animals; D001369:Axons / Q000378:metabolism / Q000473:pathology; D001921:Brain / Q000196:embryology* / Q000378:metabolism* / Q000473:pathology; D004195:Disease Models, Animal; D000071058:Huntingtin Protein / Q000378:metabolism*; D006816:Huntington Disease / Q000196:embryology* / Q000378:metabolism* / Q000473:pathology; D007150:Immunohistochemistry; D008822:Mice, Transgenic; D066263:Protein Aggregation, Pathological / Q000196:embryology / Q000378:metabolism / Q000473:pathology
chemical_list: C506812:Htt protein, mouse; D000071058:Huntingtin Protein
keywords: Huntington’s disease; aggregation; development; mouse models