TMPRSS3

TMPRSS3

Target · Transmembrane serine protease

TMPRSS3 Transmembrane serine protease 3 · transmembrane protease, serine 3

TMPRSS3 (transmembrane serine protease 3) is a probable type II transmembrane serine protease of the peptidase S1 family that plays a role in hearing. It is also known by the aliases DFNB10 and DFNB8, reflecting its link to autosomal recessive deafness. Browse Assay Genie's current range of TMPRSS3 research reagents below, or read on for the biology behind the target.

Endoplasmic reticulum membranePeptidase S1 serine proteaseLinked to recessive deafness
454 aa
Protein length
~49 kDa
Molecular weight
21q22.3
Gene locus
EC 3.4.21.-
Enzyme class
Target information

What is TMPRSS3 (transmembrane serine protease 3)?

TMPRSS3 is a probable serine protease that plays a role in hearing. It acts as a permissive factor for cochlear hair cell survival and activation at the onset of hearing, and it is required for saccular hair cell survival. In vitro, TMPRSS3 activates the epithelial sodium channel ENaC. The protein is annotated to the endoplasmic reticulum membrane.

The TMPRSS3 gene encodes a protein of the peptidase S1 family, classed by HGNC within the type II transmembrane serine proteases and the scavenger receptor cysteine rich domain containing group. The encoded protein contains a serine protease domain, a transmembrane domain, an LDL receptor-like domain and a scavenger receptor cysteine-rich domain. Alternatively spliced transcript variants have been described. The gene is expressed in foetal cochlea and many other tissues, and is thought to be involved in the development and maintenance of the inner ear, or of the contents of the perilymph and endolymph. The mouse ortholog is Tmprss3.

The aliases DFNB10 and DFNB8 come from the identification of the gene through its association with both congenital and childhood onset autosomal recessive deafness. Serine proteases are involved in a variety of biological processes, and their malfunction often leads to human diseases and disorders. TMPRSS3 has also been identified as a tumour-associated gene that is overexpressed in ovarian tumours, which makes it of interest beyond auditory research. Researchers studying the TMPRSS3 protein, from antibody-based detection to ELISA-based quantification, typically work in hearing biology or cancer research.

Hearing and deafness research

TMPRSS3 was identified through congenital and childhood onset autosomal recessive deafness and supports cochlear hair cell survival at the onset of hearing.

Inner ear development

The gene is expressed in foetal cochlea and is thought to contribute to development and maintenance of the inner ear and its fluid contents.

Ovarian tumour biology

TMPRSS3 is a tumour-associated gene overexpressed in ovarian tumours, making it a candidate for studies of protease involvement in cancer.

Protease and ion channel studies

As a serine protease that activates ENaC in vitro, TMPRSS3 is relevant to work on protease regulation of ion channels.

Background

TMPRSS3 frequently asked questions

What does TMPRSS3 do?

TMPRSS3 is a probable serine protease that plays a role in hearing. It acts as a permissive factor for cochlear hair cell survival and activation at the onset of hearing, and is required for saccular hair cell survival. It also activates ENaC in vitro.

Why is TMPRSS3 also called DFNB10 and DFNB8?

The gene was identified through its association with both congenital and childhood onset autosomal recessive deafness. The aliases DFNB10 and DFNB8 reflect these deafness loci. Its former name was transmembrane protease, serine 3, and the current HGNC name is transmembrane serine protease 3.

Is TMPRSS3 linked to disease other than deafness?

Yes. Besides its association with autosomal recessive deafness, TMPRSS3 was identified as a tumour-associated gene overexpressed in ovarian tumours. The mouse ortholog is Tmprss3; check the datasheet of any product for its validated reactivity.

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Related targets

Other type II transmembrane serine proteases with products at Assay Genie.

Gene and protein annotations from HGNC, NCBI Gene and UniProt P57727; product specifications from Assay Genie datasheets.

TMPRSS3 Product listing