Aziz M. Ulug1,
An Vo1, Miklos Argyelan1, Wynne K. Schiffer1,
Lauren Tanabe2, William T. Dauer3, David Eidelberg1
1The Feinstein Institute for Medical
Research, Manhasset, NY, United States; 2Columbia UNiversity, New
York, United States; 3University of Michigan Medical School, Ann
Arbor, MI, United States
Dystonia
is a neurological disease characterized by sustained involuntary muscle
contractions. Eight DYT1 heterozygous
mice and six littermate controls using invivo MRI, FDG microPET and exvivo
MRDTI were studied. We compared
changes in mouse genecopy of DYT1 dystonia with their wild type littermate
controls. We found that DYT1 mice
exhibit metabolic and FA abnormalities that resemble the DYT1 patients. The correlation between striatum and
cerebellum metabolic activity and the correlation between the metabolic
activity and the FA abnormalities are also shown.