Each of us carries about half a dozen defective genes, which we remain unaware of unless we suffer from a genetic disease. However, approximately one in ten people will suffer from an inherited genetic disorder. The ever developing technique of gene therapy replaces a faulty gene with a working gene, to restore function to the cell.
Researchers at the University of Virginia Health have discovered a way to transfer genes, which they hope will restore hearing, into diseased tissue of the human inner ear. This involved the scientists targeting the gene KCNQ4, which when mutated is known to cause genetic hearing loss in humans. They engineered a correct form of the gene and created a gene therapy delivery system that successfully transferred the gene into human hair cells that had been harvested from the inner ears of patients with hearing loss. This involved using a virus as the cloning vector to transform the genes into the hair cells where they could be expressed.
In normal functioning ears hair cells within the cochlea convert sound into electrical signals, which are ultimately transmitted to the brain. Whereas people with hearing loss suffer from too few, damaged or missing hair cells. Gene therapy is used to regenerate these hair cells. This critically important step forward brings scientists closer to curing genetic or acquired hearing loss.
Source
Related information on gene therapy
http://www.accessexcellence.org/RC/AB/BA/Gene_Therapy_Overview.php
http://www.audiologyonline.com/theHearingJournal/pdfs/hj2008_06_p46.pdf
http://www.accessexcellence.org/RC/AB/BA/Gene_Therapy_Overview.php
http://www.audiologyonline.com/theHearingJournal/pdfs/hj2008_06_p46.pdf
Kaitlin Newman
