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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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HTRA3
HtrA serine peptidase 3
Chromosome 4 · 4p16.1
NCBI Gene: 94031Ensembl: ENSG00000170801.11HGNC: HGNC:30406UniProt: P83110
59PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Protease
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
endopeptidase activityprotein bindingserine-type peptidase activityidentical protein bindingplacenta praeviaalcohol drinkingmixed connective tissue diseaseexostosis
✦AI Summary

HTRA3 is a serine protease that functions as a multifaceted regulator of cellular homeostasis and tissue remodeling. As a serine endopeptidase, HTRA3 cleaves extracellular matrix components including decorin, biglycan, and fibronectin, and negatively regulates transforming growth factor-beta (TGF-β) signaling 1. The protein exists as two isoforms generated through alternative splicing: a long form with a PDZ domain and a short form lacking this domain 2. Structurally, HTRA3 forms trimers through PDZ domain-mediated interactions, though the PDZ domain is dispensable for proteolytic activity 3. Beyond ECM remodeling, HTRA3 interacts with cytoskeletal proteins including actin, tubulin, and vimentin, exhibiting chaperone-like co-chaperone activity 4. Clinically, HTRA3 demonstrates tumor suppressor functions across multiple cancer types. In cardiac tissue, pressure overload downregulates HTRA3 expression, permitting TGF-β signaling activation that drives both fibrosis and heart failure; HTRA3 overexpression ameliorates cardiac dysfunction 1. Conversely, HTRA3 is significantly downregulated in endometrial cancer, ovarian cancer, and head and neck squamous cell carcinoma, with reduced expression correlating with increased tumor grade and poor prognosis 5, 6, 7. In ovarian physiology, HTRA3 expression increases during folliculogenesis and luteinization, suggesting developmental roles 2. The loss of HTRA3 expression in malignancies combined with its pro-apoptotic and TGF-β-inhibitory functions positions it as a promising therapeutic target.

Sources cited
1
As a serine endopeptidase, HTRA3 cleaves extracellular matrix components including decorin, biglycan, and fibronectin, and negatively regulates transforming growth factor-beta (TGF-β) signaling .
PMID: 35672400
2
The protein exists as two isoforms generated through alternative splicing: a long form with a PDZ domain and a short form lacking this domain .
PMID: 20540986
3
Structurally, HTRA3 forms trimers through PDZ domain-mediated interactions, though the PDZ domain is dispensable for proteolytic activity .
PMID: 26110759
4
Beyond ECM remodeling, HTRA3 interacts with cytoskeletal proteins including actin, tubulin, and vimentin, exhibiting chaperone-like co-chaperone activity .
PMID: 29477555
5
Conversely, HTRA3 is significantly downregulated in endometrial cancer, ovarian cancer, and head and neck squamous cell carcinoma, with reduced expression correlating with increased tumor grade and poor prognosis , , .
PMID: 19424634
6
Conversely, HTRA3 is significantly downregulated in endometrial cancer, ovarian cancer, and head and neck squamous cell carcinoma, with reduced expression correlating with increased tumor grade and poor prognosis , , .
PMID: 18241672
7
Conversely, HTRA3 is significantly downregulated in endometrial cancer, ovarian cancer, and head and neck squamous cell carcinoma, with reduced expression correlating with increased tumor grade and poor prognosis , , .
PMID: 37842043
Disease Associationsⓘ20
placenta praeviaOpen Targets
0.33Weak
alcohol drinkingOpen Targets
0.32Weak
exostosisOpen Targets
0.32Weak
mixed connective tissue diseaseOpen Targets
0.32Weak
carpal tunnel syndromeOpen Targets
0.18Weak
subarachnoid hemorrhageOpen Targets
0.17Weak
osteoarthritis, hipOpen Targets
0.14Weak
mononeuropathyOpen Targets
0.13Weak
nerve plexus diseaseOpen Targets
0.13Weak
gastric cancerOpen Targets
0.07Suggestive
neoplasmOpen Targets
0.07Suggestive
preeclampsiaOpen Targets
0.06Suggestive
cancerOpen Targets
0.06Suggestive
asphyxiaOpen Targets
0.06Suggestive
HypoxemiaOpen Targets
0.06Suggestive
non-small cell lung carcinomaOpen Targets
0.06Suggestive
poisoningOpen Targets
0.05Suggestive
esophageal squamous cell carcinomaOpen Targets
0.05Suggestive
infectionOpen Targets
0.05Suggestive
pancreatic neoplasmOpen Targets
0.05Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
SKOR2Shared pathway67%LEMD3Shared pathway50%HTRA4Shared pathway40%PGA4Shared pathway33%PGA3Shared pathway33%CAPN14Shared pathway33%
Tissue Expression6 tissues
Ovary
100%
Heart
72%
Lung
31%
Bone Marrow
17%
Liver
3%
Brain
1%
Gene Interaction Network
Click a node to explore
HTRA3SKOR2LEMD3HTRA4PGA4PGA3CAPN14
PROTEIN STRUCTURE
Preparing viewer…
PDB2P3W · 1.70 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.39LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.05 [0.80–1.39]
RankingsWhere HTRA3 stands among ~20K protein-coding genes
  • #7,781of 20,598
    Most Researched59
  • #14,402of 17,882
    Most Constrained (LOEUF)1.39
Genes detectedHTRA3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Cardiac fibroblasts regulate the development of heart failure via Htra3-TGF-β-IGFBP7 axis.
PMID: 35672400
Nat Commun · 2022
1.00
2
Structural and Functional Analysis of Human HtrA3 Protease and Its Subdomains.
PMID: 26110759
PLoS One · 2015
0.90
3
High-temperature requirement factor A3 (Htra3): a novel serine protease and its potential role in ovarian function and ovarian cancers.
PMID: 20540986
Mol Cell Endocrinol · 2010
0.80
4
Expression of human HtrA1, HtrA2, HtrA3 and TGF-beta1 genes in primary endometrial cancer.
PMID: 19424634
Oncol Rep · 2009
0.70
5
Serine proteases HTRA1 and HTRA3 are down-regulated with increasing grades of human endometrial cancer.
PMID: 16650464
Gynecol Oncol · 2006
0.60