NEW FLY MODEL FOR NEURODEGENERATIVE DISEASES
Juan Botas and colleagues at the Baylor College of Medicine, Houston, Texas, (Nature, Vol. 408, No. 6808, 02 Nov 2000) have developed a new fly model of the human neurodegenerative disorder Spinocerebellar ataxia type 1 (SCA1). They have engineered fruit flies to express either the normal human ataxin-1 protein, or a mutated form of the protein with expanded repeats of the amino acid glutamine, in the fly nervous system. The flies with the mutant protein have the same abnormalities as are seen in SCA1.
Botas and colleagues used this model to identify new proteins involved in the pathogenesis of SCA1. Mutations that affected the development of the disease were found in genes encoding proteins involved in the control of protein folding, in the clearance of misfolded proteins, cellular detoxification, the control of gene expression, and the control of the subcellular localization of proteins.
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