![]() ![]() At Boston College (where I received my undergraduate, Master’s, and Ph.D., all in the biological sciences) I sat in the front seat of my classes, as close to the speaker as possible. I continue to use a hearing aid in my right ear, and thankfully for the past eight years, my hearing has remained stable, if stably poor. In graduate school I began using hearing aids and later received a cochlear implant in my left ear. Over time my hearing loss became bilateral and progressive, and its cause remains unknown. In grade school, I simply tilted my right ear toward sound sources. Managing the loss of a primary sense is all about adaptation. The audiologist explained to me that as a result, I may not be able to hear birds singing as easily, and that I may need to concentrate more to understand words starting with “sh,” “k,” or “t.” Sensing my alarm, she tried to reassure me by saying it was unlikely that the hearing loss would affect both ears, and if it did, it would likely not be to the same extent. I was about 9 when hearing loss in my left ear was first detected. For more, see her Biochemistry paper:, “The Usher Syndrome Type IIIB Histidyl-tRNA Synthetase Mutation Confers Temperature Sensitivity. Susan Robey-Bond, Ph.D., was a 2012 Emerging Research Grants recipient. ![]() How these proteins are implicated in the hearing processes will eventually help develop cures or better treatments for Usher syndrome. More specifically, cells from patients containing the Y454S mutation displayed lower levels of protein synthesis, which could explain the onset of deafness these patients experience. In later analysis, though, the team found that at an elevated temperature the Y454S substitution was less stable than the wild-type. The amino acid activation, aminoacylation, and tRNA binding functions were all consistent between the mutation and wild-type genes. The team studying the biochemical properties of the gene compared the Y454S mutation in the HARS enzyme with its wild-type (non-mutated) form and found similar functional biochemical characteristics, as stated in the researchers’ recent paper in Biochemistry. It is caused by a USH3B mutation, which substitutes a serine amino acid for a tyrosine amino acid in HARS.
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