Defective Tmprss3-Associated Hair Cell Degeneration in Inner Ear Organoids

The role of TMPRSS3 in hair cell (HC) remains unclear. Nelson and colleagues generated stem cell-derived inner ear organoids with Tmprss3-mutations to show that 1) inner ear organoids exhibit comparative effects of the genetic abnormality to its in vivo counterparts, 2) Tmprss3-mutations lead to apoptosis and reduced BK channels in HCs, and 3) cell membrane-bound TMPRSS3 is localized in HCs. Overall design: Single-cell RNA sequencing (scRNA-Seq) was performed with day 25 and day 35 organoids from R1/E (WT) and R1/E-Tmprss3KO-GFPKI (KO).