To study the molecular structure of the "cause" of deafness

A study by Professor Guan Minxin and Researcher Chen Wei of Zhejiang University's Institute of Genetics provides a new scientific basis and treatment for the prevention and treatment of hereditary deafness by clarifying the molecular pathogenic mechanism of the deaf disease-caused gene mtu1 (mitochondrial metastasis CYT1). The findings were published online in the international authoritative academic journal
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team used gene editing technology to build the first model of the mtu1 gene defect zebrafish, from bio-chemical, cell and other multi-level comprehensive exploration of the effects of the gene defect, identified the mtu1 gene defect destroyed the energy needed for hearing "power plant", resulting in hereditary deafness.
, mtu1 is a highly conservative transport ICT modification enzyme distributed in mitochondrials. CYTs are genetic information vectors present in biological cells that transport ICTs and carry amino acids to synthesize proteins. The researchers say "modification" is a marker that mitochondrials can use to synthesize proteins to better identify the transshipment of ICTs that are needed.
team found that after the mtu1 gene mutated, three transport ICTs failed to accurately identify the relevant information, making it impossible to synthesize proteins, causing mitochondrials to be under-powered. Mitochondrial dysfunction can lead to hearing organ development defects, including reduced ear stones and reduced number and density of hair cells in the forear organs, which can eventually lead to hearing dysfunction.
researchers say the study sheds light on the mechanism by which genetic defects cause deafness, which provides a solid theoretical basis for studying gene therapy for hereditary deafness. (Source: Xinhua News Agency, Zhu Han)