TMPRSS3

Chr 21AR

transmembrane serine protease 3

Also known as: DFNB10, DFNB8, ECHOS1, TADG12

This gene encodes a protein that belongs to the serine protease family. The encoded protein contains a serine protease domain, a transmembrane domain, an LDL receptor-like domain, and a scavenger receptor cysteine-rich domain. Serine proteases are known to be involved in a variety of biological processes, whose malfunction often leads to human diseases and disorders. This gene was identified by its association with both congenital and childhood onset autosomal recessive deafness. This gene is expressed in fetal cochlea and many other tissues, and is thought to be involved in the development and maintenance of the inner ear or the contents of the perilymph and endolymph. This gene was also identified as a tumor-associated gene that is overexpressed in ovarian tumors. Alternatively spliced transcript variants have been described. [provided by RefSeq, Jan 2012]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Deafness, autosomal recessive 8/10MIM #601072
AR

Clinical highlights

Gene-disease validity (ClinGen)
nonsyndromic genetic hearing loss · ARDefinitivesufficient evidence for diagnostic panels
0
Active trials
11
Pubs (1 yr)
P/LP submissions
P/LP missense
1.12
LOEUF
Multiple*
Mechanism· predicted
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.12LOEUF
pLI 0.000
Z-score 1.10
OE 0.76 (0.531.12)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.57Z-score
OE missense 1.10 (1.001.21)
284 obs / 258.4 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.76 (0.531.12)
00.351.4
Missense OE?1.10 (1.001.21)
00.61.4
Synonymous OE?1.25
01.21.6
LoF obs/exp: 19 / 24.9Missense obs/exp: 284 / 258.4Syn Z: -2.12

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TMPRSS3 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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