Separating Muscle Damage From Neurodegeneration In Kennedy D

Kennedy disease is a neurodegenerative disorder caused by mutations in the gene encoding the receptor for testosterone, the androgen receptor (AR). Although it is characterized by muscle weakness caused by neurodegeneration, individuals with Kennedy disease often first suffer from muscle cramps. How the diseases progresses from muscle cramps to neuromuscular weakness is not clear. So, Andrew Lieberman and colleagues from the University of Michigan generated mice expressing the mutated form of human AR (AR113Q mice). In their study, which appears online on September 14 in advance of publication in the October print issue of the Journal of Clinical Investigation, male AR113Q mice were shown to have similar characteristics to individuals with Kennedy disease. Importantly, the muscles of male AR113Q mice became damaged before the neurodegenerative process began. One consequence of this early damage to the muscles was decreased expression of a number of soluble factors that support the survival of neurons. This study indicates that the initial damage to muscle cells might be one cause of the neurodegeneration that characterizes Kennedy disease. The authors therefore suggest that therapeutics targeting this initial muscle damage might provide benefit to individuals with Kennedy disease.

###

JCI table of contents: September 14, 2006

TITLE: Androgen-dependent pathology demonstrates myopathic contribution to the Kennedy disease phenotype in a mouse knock-in model

AUTHOR CONTACT:
Andrew P. Lieberman University of Michigan Medical School, Ann Arbor, Michigan, USA.

View the PDF of this article

Contact: Karen Honey
Journal of Clinical Investigation
(责任编辑：泉水)

搜索

热门标签:

Separating Muscle Damage From Neurodegeneration In Kennedy D