Androgen receptor and Kennedy disease/spinal bulbar muscular atrophy

Kennedy Disease/Spinal Bulbar Muscular Atrophy (KD/SBMA) is a progressive neurodegenerative disease caused by genetic polyglutamine expansion of the androgen receptor. We have recently found that overexpression of wildtype androgen receptor in skeletal muscle of transgenic mice results in a KD/SBMA...

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Bibliographic Details
Published in:Hormones and behavior 2008-05, Vol.53 (5), p.729-740
Main Authors: Monks, Douglas Ashley, Rao, Pengcheng, Mo, Kaiguo, Johansen, Jamie Ann, Lewis, Gareth, Kemp, Michael Quentin
Format: Article
Language:English
Subjects:
Androgens
Animals
Axonopathy
Genotype & phenotype
Humans
Muscle, Skeletal - physiology
Muscular Atrophy, Spinal - pathology
Muscular Atrophy, Spinal - physiopathology
Myopathy
Neurological disorders
Neuromuscular systems
Neurons - physiology
Polyglutamine disease
Receptors, Androgen - physiology
Rodents
Sexual dimorphism
Testosterone
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Description
Summary:Kennedy Disease/Spinal Bulbar Muscular Atrophy (KD/SBMA) is a progressive neurodegenerative disease caused by genetic polyglutamine expansion of the androgen receptor. We have recently found that overexpression of wildtype androgen receptor in skeletal muscle of transgenic mice results in a KD/SBMA phenotype. This surprising result challenges the orthodox view that KD/SBMA requires expression of polyglutamine expanded androgen receptor within motoneurons. Theories relating to the etiology of this disease drawn from studies of human patients, cellular and mouse models are considered with a special emphasis on potential myogenic contributions to as well as the molecular etiology of KD/SBMA.
ISSN:0018-506X
1095-6867
DOI:10.1016/j.yhbeh.2007.12.009