About Beethoven and Other Mice
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The mouse project is part of the National Genome Research Network, in which the cooperation of scientists from all disciplines is encouraged. Physicians and gene researchers, all of them can learn a lot from the sick mice in the mouse clinic. Such as from the Beethoven mouse who, like its famous eponym, is deaf. The Beethoven mouse had failed the hearing test. When the scientists searched for an answer as to why this mouse was not able to hear, they discovered that the hair cells of the inner ear were no longer present. Such hair cells are needed to capture sound waves.
The deafness of the Beethoven mouse is hereditary because the hair cells degenerate in the descendents as well. To trace the cause of the disease it was therefore necessary to search for a mutation in the DNA. A genuine Sisyphos work, for each mouse has approximately 25,000 genes.
But the gene researchers were successful. They found the mutation in a gene named Tmc1. Tmc1 has the blueprint for a protein, that is in the membrane of the hair cells.
Are these research findings transferable to humans as well? In many cases the causes of disease in mouse and man are very similar, for the DNA of man and mouse is almost 95 percent identical. Families were studied in which loss of hearing developed in advanced age. And indeed: many of these families had mutations in the human Tcm1 gene.
Thanks to the Beethoven mouse, another cause for deafness is now known and new treatment possibilities can be targeted.
Page 2: Spotlessly clean rooms for the mice.
