It is not clear how common CIB2 mutations are in the US population, Ahmed says theĮarly results of these experiments are intriguing. Gene to baby mice that have the mutated version. Therapy, using a harmless virus to deliver a copy of the normal CIB2

The scientists are also experimenting with gene In the first few weeks after birth, these mice, which are born deaf, They suspect that if this modification occurs Mice, they are using the gene editing tool CRISPR to modifyĭysfunctional CIB2 genes. In addition, they areĮxploring potential therapies for CIB2-related hearing problems. Ahmed and his colleagues are now looking for other moleculesīeyond CIB2 that play a key role in hearing. Signals in the inner ear,”said the study’s co-senior author, Gregory Frolenkov, PhD, of the Department of Physiology at the University of Kentucky.ĭr. The molecular machinery that converts sound waves into electrical “This is a big step in determining the identity of key components of Human CIB2 mutation into the mouse, they found that the mice were deaf. People with this mutation cannot turn soundwaves into signals theīrain can interpret, and so are deaf. Other proteins, TMC1 and TMC2, which are crucial for converting sound toĮlectrical signals, a process known as mechanotransduction. Mutation affects the ability of the CIB2 protein to interact with two In this study, they genetically engineered mice without CIB2, as well as mice in which a human CIB2 Since then, they have studied the protein inįlies, mice, zebrafish and humans. SOM, along with their collaborators, discovered five years ago that CIB2 In the Department of Otorhinolaryngology-Head and Neck Surgery at UM Ahmed and his colleague Saima Riazuddin, PhD, Professor Each ear contains 18,000 hair cells thatĭr. Small, less than a half a micrometer in diameter, which is about the “This tells us something new about the fundamental biology of how hearing works on a molecular level.”ĬIB2, which is short for calcium and integrin-binding protein 2, isĮssential for the structure of stereocilia, the structures at the top of “We are very excited by these results', said the senior author of the study, Zubair Ahmed, PhD, Professor in the Department of Otorhinolaryngology-Head and Neck Surgery at UM SOM. The study is the first to illuminate in detail how the protein, known as CIB2, enables hearing. The findings were published recently in the Nature Communications. The new understandingĬould eventually help repair or prevent hearing problems for those with Process that turns sound waves into brain signals. Now, the new UM SOM study, has identified a crucial protein in the Of what causes hearing disorders, especially those that may have genetic In particular, with 74,000 children currently affected by profound,Įarly-onset deafness, scientists have been trying to solve the mystery Loss – a condition that affects some 40 million Americans. Scientists repair hearing problems or protect those at risk for hearing Researchers at the University of Maryland School of Medicine (UM SOM)Īnnounced a breakthrough discovery that could eventually help

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