TSPEAR

Chr 21

thrombospondin type laminin G domain and EAR repeats

Also known as: C21orf29, DFNB98, ECTD14, STHAG10, TSP-EAR

This gene encodes a protein that contains a N-terminal thrombospondin-type laminin G domain and several tandem arranged epilepsy-associated repeats (EARs). A mutation in this gene is the cause of autosomal recessive deafness-98. Alternate splicing results in multiple transcript variants. [provided by RefSeq, Dec 2012]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtDeafness, autosomal recessive, 98
UniProtEctodermal dysplasia 14, hypohidrotic/hair/tooth/nail type
UniProtTooth agenesis, selective, 10

Clinical highlights

Gene-disease validity (ClinGen)
nonsyndromic genetic hearing loss · ARDisputedevidence questions this relationship
0
Active trials
13
Pubs (1 yr)
P/LP submissions
P/LP missense
1.20
LOEUF
Mechanism
📖
GeneReview available — TSPEAR
Authoritative clinical overview · Recommended first read
Open GeneReview ↗
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.20LOEUF
pLI 0.000
Z-score 0.67
OE 0.87 (0.651.20)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-0.95Z-score
OE missense 1.13 (1.051.22)
473 obs / 418.2 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.87 (0.651.20)
00.351.4
Missense OE?1.13 (1.051.22)
00.61.4
Synonymous OE?1.05
01.21.6
LoF obs/exp: 28 / 32.1Missense obs/exp: 473 / 418.2Syn Z: -0.54

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TSPEAR · 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.

Search ClinicalTrials.gov →