Discovery Sheds Light on Patients with Muscular Dystrophy

March 30, 2007

Recently, a newly developed curcumin compound has proven effective against muscular dystrophy in tests on rodents, conducted by a US-based research team led by Professor Chawnshang Chang from George H. Whipple Laboratory for Cancer Research, University of Rochester Medical Center, NY. As a first author, Dr. Zhiming Yang, one of the researchers from the Second Affiliated Hospital, Zhejiang University School of Medicine, published an article entitled ASC-J9 ameliorates spinal and bulbar muscular atrophy phenotype via degradation of androgen receptor, on Journal Nature Medicine.

X-linked spinal and bulbar muscular atrophy (SBMA or Kennedy disease) is an inherited neurodegenerative disorder caused by the expansion of the polyglutamine tract of the androgen receptor (AR-polyQ). The length of the AR-polyQ tract is inversely correlated with the age of SBMA onset. The effects of the disease are only seen in males, as female carriers are usually asymptomatic. Characteristics of SBMA include proximal muscular atrophy, weakness, contraction fasciculation and bulbar involvement. The nuclear inclusions containing AR-polyQ in the residual motor neurons of the brain stem, spinal cord and other visceral organs are considered to be relevant to the pathophysiology of this disease.

ASC-J9, a curcumnoid extracted from ginger and curry, was used to treat some 60 mice affected by SBMA. The research team from the University of Rochester Medical Center, New York, found that the SBMA mice showed normal sexual activity and improved fertility, suggesting that this strategy might provide a better approach to treating SBMA in men. Dr. Zhiming Yang from Zhejiang University and Dr. Yujia Chang from Taipei Medical University and Hospital, contributed equally to this discovery.

(Second Affiliated Hospital, School of Medicine)