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Human Molecular Genetics, 2002, Vol. 11, No. 21 2657-2672
© 2002 Oxford University Press

Suppression of polyglutamine toxicity by a Drosophila homolog of myeloid leukemia factor 1

Parsa Kazemi-Esfarjani1,* and Seymour Benzer2

1Department of Physiology and Biophysics, Center for Neuroscience, School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, NY 14214, USA and 2California Institute of Technology, Division of Biology, Pasadena, CA 91125, USA

Received July 22, 2002; Accepted July 31, 2002

The toxicity of an abnormally long polyglutamine [poly(Q)] tract within specific proteins is the molecular lesion shared by Huntington's disease (HD) and several other hereditary neurodegenerative disorders. By a genetic screen in Drosophila, devised to uncover genes that suppress poly(Q) toxicity, we discovered a Drosophila homolog of human myeloid leukemia factor 1 (MLF1). Expression of the Drosophila homolog (dMLF) ameliorates the toxicity of poly(Q) expressed in the eye and central nervous system. In the retina, whether endogenously or ectopically expressed, dMLF co-localized with aggregates, suggesting that dMLF alone, or through an intermediary molecular partner, may suppress toxicity by sequestering poly(Q) and/or its aggregates.

* To whom correspondence should be addressed: Tel: +1 7168293290; Fax: +1 7168293050; Email: pkazemi{at}acsu.buffalo.edu


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