TMPRSS3

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TMPRSS3

Notation for the gene for DFNB10.

TMPRSS3

A gene on chromosome 21q22.3 that encodes a putative serine protease which plays a role in hearing, acting as a permissive factor for cochlear hair cell survival and activation at the onset of hearing; it is required for saccular hair cell survival. TMPRSS3 activates the epithelial sodium channel (ENaC). It is also a less preferred gene symbol for what is now designated as TMPRSS4, see there. 

Molecular pathology
Defects in TMPRSS3 cause deafness autosomal recessive types 8 and 10.