Article · Wikipedia archive · Last revised Jun 22, 2026

Serine protease HTRA1

Serine protease HTRA1 is an enzyme that in humans is encoded by the HTRA1 gene. The HTRA1 protein is composed of four distinct protein domains. They are from amino-terminus to carboxyl-terminus an Insulin-like growth factor binding domain, a kazal domain, a trypsin-like peptidase domain and a PDZ domain.

Last revised
Jun 22, 2026
Read time
≈ 4 min
Length
1,014 w
Citations
11
Source
HTRA1
Available structures
PDBOrtholog search: PDBe RCSB
Identifiers
AliasesHTRA1, ARMD7, CARASIL, HtrA, L56, ORF480, PRSS11, CADASIL2, HtrA serine peptidase 1
External IDsOMIM: 602194; MGI: 1929076; HomoloGene: 31114; GeneCards: HTRA1; OMA:HTRA1 - orthologs
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_002775

NM_019564

RefSeq (protein)

NP_002766

NP_062510

Location (UCSC)Chr 10: 122.46 – 122.51 MbChr 7: 130.54 – 130.59 Mb
PubMed search34
Wikidata
View/Edit HumanView/Edit Mouse

Serine protease HTRA1 is an enzyme that in humans is encoded by the HTRA1 gene.56 The HTRA1 protein is composed of four distinct protein domains. They are from amino-terminus to carboxyl-terminus an Insulin-like growth factor binding domain, a kazal domain, a trypsin-like peptidase domain and a PDZ domain.

This gene encodes a member of the trypsin family of serine proteases. This protein is a secreted enzyme that is proposed to regulate the availability of insulin-like growth factors (IGFs) by cleaving IGF-binding proteins. It has also been suggested to be a regulator of cell growth.6

Mutations of this gene are responsible for the development of CARASIL, a genetic form of cerebral vasculopathy.

References

References

  1. GRCh38: Ensembl release 89: ENSG00000166033Ensembl, May 2017
  2. GRCm38: Ensembl release 89: ENSMUSG00000006205Ensembl, May 2017
  3. "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. Zumbrunn J, Trueb B (Jan 1997). "Primary structure of a putative serine protease specific for IGF-binding proteins". FEBS Lett. 398 (2–3): 187–92. doi:10.1016/S0014-5793(96)01229-X. PMID 8977104. S2CID 39934612.
  6. "Entrez Gene: HTRA1 HtrA serine peptidase 1".
External links
Further reading

Further reading