Modeling Neurodegenerative Diseases in vivo

Huntington's Disease - New Perspectives Based on Neuroendocrine Changes in Rodent Models
Åsa Petersén^a, Sofia Hult^a, Deniz Kirik<sup>b

a</sup>Translational Neuroendocrine Research Unit, and
^bBrain Repair and Imaging in Neural Systems (BRAINS), Department of Experimental Medical Science, Lund University, Lund, Sweden

Address of Corresponding Author

Neurodegenerative Dis 2009;6:154-164 (DOI: 10.1159/000225377)

  Key Words

• Huntington's disease
• Neuroendocrine
• Mouse
• Rat
• Hypothalamus
• Model

  Abstract

Huntington's disease (HD) is a neurodegenerative disorder caused by an expanded CAG repeat in the huntingtin gene. Although it is characterized by progressive motor impairments, cognitive changes and psychiatric disturbances are major components of the disease. In addition, recent studies have shown that other non-motor symptoms such as alterations in sleep pattern, disruption of the circadian rhythm and increased energy metabolism are common and occur early. Emerging evidence suggests that the latter symptoms are likely results of disturbed functions of the hypothalamus and neuroendocrine circuits, which are known to be central in the regulation of emotion, sleep and metabolism. Whereas clinical data are essential to define key pathological features of HD, animal models that can recapitulate the neurobiological and behavioral features of the disorder are critical tools to elucidate the underlying pathogenic mechanisms. Recent studies employing different HD rodent models have been instrumental in identifying a number of neuroendocrine alterations as well as in highlighting novel potential disease pathways. This review summarizes the current state of knowledge derived from neuroendocrine studies in rodent models of HD in light of clinical relevance and points to future implications for this emerging field.

Copyright © 2009 S. Karger AG, Basel

  Author Contacts

Assoc. Prof. Åsa Petersén, MD, PhD
Translational Neuroendocrine Research Unit
BMC D11
SE-22184 Lund (Sweden)
Tel. +46 46 222 1686, Fax +46 46 222 3436, E-Mail asa.petersen@med.lu.se

  Article Information

Received: January 11, 2009
Accepted after revision: March 11, 2009
Published online: June 12, 2009
Number of Print Pages : 11
Number of Figures : 0, Number of Tables : 3, Number of References : 126