Affiliations: [a] Department of Biochemistry and Cellular and Molecular Biology, University of Tennessee, Knoxville, TN, USA
| [b] Department of Pediatrics, Vanderbilt University Medical Center, Nashville, TN, USA
| [c] Department of Pediatrics, Neurology, and Biochemistry, Vanderbilt University (VU) and VU Medical Center, Nashville, TN, USA
| [d] Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, London, UK
Correspondence to: Alexander P. Osmand, Department of Biochemistry and Cellular and Molecular Biology, University of Tennessee, Knoxville, TN 37996, USA. Tel.: +1 865 974 4079; Fax: +1 865 974 6306; E-mail: [email protected].
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.
Keywords: Huntington’s disease, mouse models, aggregation, development