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rRNA processing:

Ribosomal RNA is a component of the ribosome, the cell's protein-making factory. Without ribosomes, all enzymatic reactions come to a halt and the cell dies eventually. Even though ribosome is so important to the survival of a cell, its biogenesis is still not yet completely understood. dribble gives us a chance to study this important and intriguing question. The dribble gene encodes a KH RNA-binding domain protein in Drosophila. When mutated, dribble mutant flies display homozygous lethality during first instar larval stage of developement. When homozygous dribble mutant larvae were examined, a ribosomal RNA cleavage defect was observed. We are interested in knowing what role does dribble play in ribosomal RNA processing. Through these studies, we hope to understand better the biogenesis of ribosomes.

Human genetic diseases:

Using an established Drosophila transgenic model of Machado Joseph Disease (MJD), we study the pathogenesis of polyglutamine disease. Previously, it was shown that flies expressing the MJD disease protein, as in MJD patients, developed late-onset, progressive neuronal degeneration.

Molecular chaperones, such as Hsp70, had previously been shown to be potent suppressors of neurodegeneration. Not only polyglutamine disease, Hsp70 has also been shown to be a powerful suppressor of Parkinson-like pathology in flies. This and other findings suggest different degenerative brain diseases may have certain core overlapping pathogenic pathways. In LDR, we sought to identify genes that are involved in these disease pathogenic pathways. We are also interested in elucidating the underlying molecular mechanisms of disease suppression. Our long-term goal is to apply what we learn from these various different disease models to patients with neurodegenerative diseases as a basis for therapeutic or preventive medicine.