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Juntendo University research: Step forward towards treating hereditary deafness

Researchers at Juntendo University report in the journal Human Molecular Genetics that a stem-cell technique may be used to develop a treatment of a common mutation-related type of deafness. The method involves reproducing cells occurring in the human inner ear where the researchers emphasize that the pathological condition could be reproduced with iPS derived from a typical patient. Illustration of the research described in this paper. Deafness is the most common sensory impairment in newborn children about 1 child in 1000 is born with hearing loss, or develops it in early childhood. Half of these cases have a genetic cause; very often, this type of deafness is related to a mutation of a gene called gap junction beta 2 (GJB2) , which encodes a protein called connexin 26 (CX26) . This protein occurs in cells in the cochlea, the part of the inner ear enabling hearing. One way of treating GJB2-related deafness would be to reproduce properly functioning cochlear cells and

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