How does inheritance hearing loss happen?

Japanese researchers have used an inducible pluripotent stem cell (ips cell) from a patient with hereditary hearing loss and recombinant human proteins to develop inner ear cells and compared them with healthy human inner ear cells, finding the pathogenesis of this disease. This study is also expected to be used to find other treatment of hearing impairment.

Goitre - deafness syndrome is a rare congenital thyroid hormone organic synthesis disorders. It is an autosomal recessive inheritance with goiter and sensorineural deafness as the main clinical symptoms. Patients tend to develop hearing impairment from an early age and may have a language delay. The abnormalities of genes which control and synthesize protein Pendrin are considered to be pathogenic, but the specific pathogenesis is unclear.

Researchers at Keio University and other institutions used patients' blood to produce ips cells, and then they were induced and differentiated into inner ear cells. The researchers compared the inner ear cells differentiated from healthy ips cells. The researchers found that the Pendrin proteins in the inner ear cells differentiated from ips cells showed abnormal agglutination. The cells are easy to die, which is similar to the pathogenesis of Alzheimer's disease.

Ips cells are stem cells transformed by somatic cells through induced factors after treatment, having the possibility of developing into a variety of tissue cells.

Researchers in the cell experiments also found that an immunosuppressive agent sirolimus can effectively inhibit the inner ear cell death. In addition, the use of ips cell culture in patients with inner ear cells are also expected to find other treatments for hearing impairment, and the researchers prepared to conduct further research on this. Flarebio provides you with superior recombinant proteins including recombinant TLR2 at competitive prices.