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Protein

Serine protease HTRA1

Gene

HTRA1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the ‘protein existence’ evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Serine protease with a variety of targets, including extracellular matrix proteins such as fibronectin. HTRA1-generated fibronectin fragments further induce synovial cells to up-regulate MMP1 and MMP3 production. May also degrade proteoglycans, such as aggrecan, decorin and fibromodulin. Through cleavage of proteoglycans, may release soluble FGF-glycosaminoglycan complexes that promote the range and intensity of FGF signals in the extracellular space. Regulates the availability of insulin-like growth factors (IGFs) by cleaving IGF-binding proteins. Inhibits signaling mediated by TGF-beta family members. This activity requires the integrity of the catalytic site, although it is unclear whether TGF-beta proteins are themselves degraded. By acting on TGF-beta signaling, may regulate many physiological processes, including retinal angiogenesis and neuronal survival and maturation during development. Intracellularly, degrades TSC2, leading to the activation of TSC2 downstream targets.3 Publications

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection describes interesting single amino acid sites on the sequence that are not defined in any other subsection. This subsection can be displayed in different sections (‘Function’, ‘PTM / Processing’, ‘Pathology and Biotech’) according to its content.<p><a href='/help/site' target='_top'>More...</a></p>Sitei169Involved in trimer stabilization1 Publication1
Sitei171Involved in trimer stabilization1 Publication1
<p>This subsection of the ‘Function’ section is used for enzymes and indicates the residues directly involved in catalysis.<p><a href='/help/act_site' target='_top'>More...</a></p>Active sitei220Charge relay system1 Publication1
Active sitei250Charge relay system1 Publication1
Sitei278Involved in trimer stabilization1 Publication1
Active sitei328Charge relay system1 Publication1

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Molecular functionGrowth factor binding, Hydrolase, Protease, Serine protease

Enzyme and pathway databases

BRENDA Comprehensive Enzyme Information System

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BRENDAi
3.4.21.108 2681

Reactome - a knowledgebase of biological pathways and processes

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Reactomei
R-HSA-1474228 Degradation of the extracellular matrix

Protein family/group databases

MEROPS protease database

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MEROPSi
S01.277

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
Recommended name:
Serine protease HTRA1 (EC:3.4.21.-)
Alternative name(s):
High-temperature requirement A serine peptidase 1
L56
Serine protease 11
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: ‘Name’, ‘Synonyms’, ‘Ordered locus names’ and ‘ORF names’.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:HTRA1
Synonyms:HTRA, PRSS11
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the ‘taxonomic identifier’ or ‘taxid’.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes_manual">proteome</a> can consist of several components. <br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 10

Organism-specific databases

Eukaryotic Pathogen Database Resources

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EuPathDBi
HostDB:ENSG00000166033.11

Human Gene Nomenclature Database

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HGNCi
HGNC:9476 HTRA1

Online Mendelian Inheritance in Man (OMIM)

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MIMi
602194 gene

neXtProt; the human protein knowledge platform

More...
neXtProti
NX_Q92743

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Cell membrane, Cytoplasm, Membrane, Secreted

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This subsection of the ‘Pathology and Biotech’ section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

Macular degeneration, age-related, 7 (ARMD7)2 Publications
Disease susceptibility is associated with variations affecting the gene represented in this entry.
Disease descriptionA form of age-related macular degeneration, a multifactorial eye disease and the most common cause of irreversible vision loss in the developed world. In most patients, the disease is manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane.
See also OMIM:610149
Cerebral arteriopathy, autosomal recessive, with subcortical infarcts and leukoencephalopathy (CARASIL)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA cerebrovascular disease characterized by non-hypertensive arteriopathy of cerebral small vessels with subcortical infarcts, alopecia, and spondylosis. Small cerebral arteries show arteriosclerotic changes, fibrous intimal proliferation, and hyaline degeneration with splitting of the intima and/or the internal elastic membrane. Neurologic features include progressive dementia, gait disturbances, extrapyramidal and pyramidal signs, and demyelination of the cerebral white matter with sparing of U fibers.
See also OMIM:600142
Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_063148252A → T in CARASIL; has 21 to 50% normal protease activity; is unable to suppress TGF-beta activity. 1 PublicationCorresponds to variant dbSNP:rs113993968EnsemblClinVar.1
Natural variantiVAR_063149297V → M in CARASIL; has 21 to 50% normal protease activity; is unable to suppress TGF-beta activity. 1 PublicationCorresponds to variant dbSNP:rs113993969EnsemblClinVar.1
Cerebral arteriopathy, autosomal dominant, with subcortical infarcts and leukoencephalopathy, 2 (CADASIL2)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA cerebrovascular disease characterized by multiple subcortical infarcts, pseudobulbar palsy, dementia, and the presence of granular deposits in small cerebral arteries producing ischemic stroke.
See also OMIM:616779
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_076373121S → R in CADASIL2. 1 Publication1
Natural variantiVAR_076374123A → S in CADASIL2. 1 Publication1
Natural variantiVAR_076375133R → G in CADASIL2. 1 Publication1
Natural variantiVAR_076376166R → L in CADASIL2; loss of proteolytic activity. 1 PublicationCorresponds to variant dbSNP:rs864622781EnsemblClinVar.1
Natural variantiVAR_076377173A → P in CADASIL2; loss of proteolytic activity. 1 PublicationCorresponds to variant dbSNP:rs781563777EnsemblClinVar.1
Natural variantiVAR_076378284S → G in CADASIL2; partial loss of proteolytic activity. 1 Publication1
Natural variantiVAR_076379284S → R in CADASIL2; loss of proteolytic activity. 1 PublicationCorresponds to variant dbSNP:rs864622782EnsemblClinVar.1
Natural variantiVAR_076380285P → Q in CADASIL2; loss of proteolytic activity. 1 Publication1
Natural variantiVAR_076381286F → V in CADASIL2; loss of proteolytic activity. 1 Publication1
Natural variantiVAR_076382450D → H in CADASIL2; unknown pathological significance; small decrease, if any, in proteolytic activity. 1 PublicationCorresponds to variant dbSNP:rs772225907Ensembl.1

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/manual/pathology_and_biotech_section">'Pathology and Biotech'</a> section describes the effect of the experimental mutation of one or more amino acid(s) on the biological properties of the protein.<p><a href='/help/mutagen' target='_top'>More...</a></p>Mutagenesisi328S → A: Loss of activity. 2 Publications1

Keywords - Diseasei

Age-related macular degeneration, Disease mutation

Organism-specific databases

DisGeNET

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DisGeNETi
5654

GeneReviews a resource of expert-authored, peer-reviewed disease descriptions.

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GeneReviewsi
HTRA1

MalaCards human disease database

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MalaCardsi
HTRA1
MIMi600142 phenotype
610149 phenotype
616779 phenotype

Open Targets

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OpenTargetsi
ENSG00000166033

Orphanet; a database dedicated to information on rare diseases and orphan drugs

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Orphaneti
199354 CARASIL
482077 HTRA1-related autosomal dominant cerebral small vessel disease
3148 Malignant peripheral nerve sheath tumor
279 NON RARE IN EUROPE: Age-related macular degeneration

The Pharmacogenetics and Pharmacogenomics Knowledge Base

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PharmGKBi
PA33829

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

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BioMutai
HTRA1

Domain mapping of disease mutations (DMDM)

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DMDMi
18202620

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section denotes the presence of an N-terminal signal peptide.<p><a href='/help/signal' target='_top'>More...</a></p>Signal peptidei1 – 22Sequence analysisAdd BLAST22
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_000002694323 – 480Serine protease HTRA1Add BLAST458

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the PTM / Processing":/help/ptm_processing_section section describes the positions of cysteine residues participating in disulfide bonds.<p><a href='/help/disulfid' target='_top'>More...</a></p>Disulfide bondi110 ↔ 130PROSITE-ProRule annotation
Disulfide bondi119 ↔ 155PROSITE-ProRule annotation

Keywords - PTMi

Disulfide bond

Proteomic databases

Encyclopedia of Proteome Dynamics

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EPDi
Q92743

jPOST - Japan Proteome Standard Repository/Database

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jPOSTi
Q92743

PaxDb, a database of protein abundance averages across all three domains of life

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PaxDbi
Q92743

PeptideAtlas

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PeptideAtlasi
Q92743

PRoteomics IDEntifications database

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PRIDEi
Q92743

ProteomicsDB human proteome resource

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ProteomicsDBi
75437

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

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iPTMneti
Q92743

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

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PhosphoSitePlusi
Q92743

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

<p>This subsection of the ‘Expression’ section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms. By default, the information is derived from experiments at the mRNA level, unless specified ‘at protein level’. <br></br>Examples: <a href="http://www.uniprot.org/uniprot/P92958#expression">P92958</a>, <a href="http://www.uniprot.org/uniprot/Q8TDN4#expression">Q8TDN4</a>, <a href="http://www.uniprot.org/uniprot/O14734#expression">O14734</a><p><a href='/help/tissue_specificity' target='_top'>More...</a></p>Tissue specificityi

Widely expressed, with strongest expression in placenta (at protein level). Secreted by synovial fibroblasts. Up-regulated in osteoarthritis and rheumatoid arthritis synovial fluids and cartilage as compared with non-arthritic (at protein level).3 Publications

<p>This subsection of the ‘Expression’ section provides information on the expression of the gene product at various stages of a cell, tissue or organism development. By default, the information is derived from experiments at the mRNA level, unless specified ‘at the protein level’.<p><a href='/help/developmental_stage' target='_top'>More...</a></p>Developmental stagei

In the placenta, in the first trimester of gestation, low expression in the cells surrounding villi both in the inner layer of the cytotrophoblast and in the outer layer of the syncytiotrophoblast (at protein level). In the third trimester of gestation, very strong expression in the outer layer forming the syncytiotrophoblast and lower in the cytotrophoblast (at protein level).1 Publication

Gene expression databases

Bgee dataBase for Gene Expression Evolution

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Bgeei
ENSG00000166033 Expressed in 234 organ(s), highest expression level in tendon

CleanEx database of gene expression profiles

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CleanExi
HS_HTRA1

ExpressionAtlas, Differential and Baseline Expression

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ExpressionAtlasi
Q92743 baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

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Genevisiblei
Q92743 HS

Organism-specific databases

Human Protein Atlas

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HPAi
HPA036655

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction_section">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

Forms homotrimers. In the presence of substrate, may form higher-order multimers in a PDZ-independent manner. Interacts with TGF-beta family members, including BMP4, TGFB1, TGFB2, activin A and GDF5 (By similarity).By similarity

<p>This subsection of the '<a href="http://www.uniprot.org/help/interaction_section%27">Interaction</a> section provides information about binary protein-protein interactions. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the <a href="http://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated on a monthly basis. Each binary interaction is displayed on a separate line.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

GO - Molecular functioni

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGrid)

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BioGridi
111635, 20 interactors

Database of interacting proteins

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DIPi
DIP-33195N

Protein interaction database and analysis system

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IntActi
Q92743, 10 interactors

Molecular INTeraction database

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MINTi
Q92743

STRING: functional protein association networks

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STRINGi
9606.ENSP00000357980

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

Secondary structure

1480
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

Select the link destinations:

Protein Data Bank Europe

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PDBei

Protein Data Bank RCSB

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RCSB PDBi

Protein Data Bank Japan

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PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2JOANMR-A380-480[»]
2YTWNMR-A370-480[»]
3NUMX-ray2.75A158-480[»]
3NWUX-ray3.20A/B/C158-375[»]
3NZIX-ray2.75A158-480[»]
3TJNX-ray3.00A/B/D161-367[»]
3TJOX-ray2.30A/B/D161-370[»]
3TJQX-ray2.00A35-156[»]

Protein Model Portal of the PSI-Nature Structural Biology Knowledgebase

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ProteinModelPortali
Q92743

SWISS-MODEL Repository - a database of annotated 3D protein structure models

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SMRi
Q92743

Database of comparative protein structure models

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ModBasei
Search...

MobiDB: a database of protein disorder and mobility annotations

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MobiDBi
Search...

Miscellaneous databases

Relative evolutionary importance of amino acids within a protein sequence

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EvolutionaryTracei
Q92743

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/family_and_domains_section">Family and Domains</a> section describes the position and type of a domain, which is defined as a specific combination of secondary structures organized into a characteristic three-dimensional structure or fold.<p><a href='/help/domain' target='_top'>More...</a></p>Domaini33 – 100IGFBP N-terminalPROSITE-ProRule annotationAdd BLAST68
Domaini98 – 157Kazal-likePROSITE-ProRule annotationAdd BLAST60
Domaini365 – 467PDZPROSITE-ProRule annotationAdd BLAST103

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Family and Domains’ section describes a region of interest that cannot be described in other subsections.<p><a href='/help/region' target='_top'>More...</a></p>Regioni204 – 364Serine proteaseAdd BLAST161

<p>This subsection of the ‘Family and domains’ section provides general information on the biological role of a domain. The term ‘domain’ is intended here in its wide acceptation, it may be a structural domain, a transmembrane region or a functional domain. Several domains are described in this subsection.<p><a href='/help/domain_cc' target='_top'>More...</a></p>Domaini

The IGFBP N-terminal domain mediates interaction with TSC2 substrate.

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Belongs to the peptidase S1C family.Curated

Keywords - Domaini

Signal

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

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eggNOGi
KOG1320 Eukaryota
COG0265 LUCA

Ensembl GeneTree

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GeneTreei
ENSGT00940000156955

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

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HOGENOMi
HOG000223641

The HOVERGEN Database of Homologous Vertebrate Genes

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HOVERGENi
HBG052044

InParanoid: Eukaryotic Ortholog Groups

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InParanoidi
Q92743

KEGG Orthology (KO)

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KOi
K08784

Identification of Orthologs from Complete Genome Data

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OMAi
PERCEPA

Database of Orthologous Groups

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OrthoDBi
630723at2759

Database for complete collections of gene phylogenies

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PhylomeDBi
Q92743

TreeFam database of animal gene trees

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TreeFami
TF323480

Family and domain databases

Integrated resource of protein families, domains and functional sites

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InterProi
View protein in InterPro
IPR009030 Growth_fac_rcpt_cys_sf
IPR000867 IGFBP-like
IPR002350 Kazal_dom
IPR036058 Kazal_dom_sf
IPR001478 PDZ
IPR036034 PDZ_sf
IPR009003 Peptidase_S1_PA
IPR001940 Peptidase_S1C

Pfam protein domain database

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Pfami
View protein in Pfam
PF00219 IGFBP, 1 hit
PF07648 Kazal_2, 1 hit

Protein Motif fingerprint database; a protein domain database

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PRINTSi
PR00834 PROTEASES2C

Simple Modular Architecture Research Tool; a protein domain database

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SMARTi
View protein in SMART
SM00121 IB, 1 hit
SM00280 KAZAL, 1 hit
SM00228 PDZ, 1 hit

Superfamily database of structural and functional annotation

More...
SUPFAMi
SSF100895 SSF100895, 1 hit
SSF50156 SSF50156, 1 hit
SSF50494 SSF50494, 1 hit
SSF57184 SSF57184, 1 hit

PROSITE; a protein domain and family database

More...
PROSITEi
View protein in PROSITE
PS51323 IGFBP_N_2, 1 hit
PS51465 KAZAL_2, 1 hit
PS50106 PDZ, 1 hit

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence_length">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>.<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequence (1+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>Sequence processingi: The displayed sequence is further processed into a mature form.

This entry has 1 described isoform and 2 potential isoforms that are computationally mapped.Show allAlign All

Q92743-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MQIPRAALLP LLLLLLAAPA SAQLSRAGRS APLAAGCPDR CEPARCPPQP
60 70 80 90 100
EHCEGGRARD ACGCCEVCGA PEGAACGLQE GPCGEGLQCV VPFGVPASAT
110 120 130 140 150
VRRRAQAGLC VCASSEPVCG SDANTYANLC QLRAASRRSE RLHRPPVIVL
160 170 180 190 200
QRGACGQGQE DPNSLRHKYN FIADVVEKIA PAVVHIELFR KLPFSKREVP
210 220 230 240 250
VASGSGFIVS EDGLIVTNAH VVTNKHRVKV ELKNGATYEA KIKDVDEKAD
260 270 280 290 300
IALIKIDHQG KLPVLLLGRS SELRPGEFVV AIGSPFSLQN TVTTGIVSTT
310 320 330 340 350
QRGGKELGLR NSDMDYIQTD AIINYGNSGG PLVNLDGEVI GINTLKVTAG
360 370 380 390 400
ISFAIPSDKI KKFLTESHDR QAKGKAITKK KYIGIRMMSL TSSKAKELKD
410 420 430 440 450
RHRDFPDVIS GAYIIEVIPD TPAEAGGLKE NDVIISINGQ SVVSANDVSD
460 470 480
VIKRESTLNM VVRRGNEDIM ITVIPEEIDP
Length:480
Mass (Da):51,287
Last modified:February 1, 1997 - v1
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:iCA20A99480FB2330
GO

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
H0Y7G9H0Y7G9_HUMAN
Serine protease HTRA1
HTRA1
221Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
A0A3B3IU24A0A3B3IU24_HUMAN
Serine protease HTRA1
HTRA1
374Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti323I → T in AAC97211 (PubMed:9852107).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_07637120A → V1 PublicationCorresponds to variant dbSNP:rs369149111EnsemblClinVar.1
Natural variantiVAR_07637251E → G1 Publication1
Natural variantiVAR_076373121S → R in CADASIL2. 1 Publication1
Natural variantiVAR_076374123A → S in CADASIL2. 1 Publication1
Natural variantiVAR_076375133R → G in CADASIL2. 1 Publication1
Natural variantiVAR_076376166R → L in CADASIL2; loss of proteolytic activity. 1 PublicationCorresponds to variant dbSNP:rs864622781EnsemblClinVar.1
Natural variantiVAR_076377173A → P in CADASIL2; loss of proteolytic activity. 1 PublicationCorresponds to variant dbSNP:rs781563777EnsemblClinVar.1
Natural variantiVAR_063148252A → T in CARASIL; has 21 to 50% normal protease activity; is unable to suppress TGF-beta activity. 1 PublicationCorresponds to variant dbSNP:rs113993968EnsemblClinVar.1
Natural variantiVAR_076378284S → G in CADASIL2; partial loss of proteolytic activity. 1 Publication1
Natural variantiVAR_076379284S → R in CADASIL2; loss of proteolytic activity. 1 PublicationCorresponds to variant dbSNP:rs864622782EnsemblClinVar.1
Natural variantiVAR_076380285P → Q in CADASIL2; loss of proteolytic activity. 1 Publication1
Natural variantiVAR_076381286F → V in CADASIL2; loss of proteolytic activity. 1 Publication1
Natural variantiVAR_063149297V → M in CARASIL; has 21 to 50% normal protease activity; is unable to suppress TGF-beta activity. 1 PublicationCorresponds to variant dbSNP:rs113993969EnsemblClinVar.1
Natural variantiVAR_076382450D → H in CADASIL2; unknown pathological significance; small decrease, if any, in proteolytic activity. 1 PublicationCorresponds to variant dbSNP:rs772225907Ensembl.1

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

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EMBLi

GenBank nucleotide sequence database

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GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

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DDBJi
Links Updated
Y07921 mRNA Translation: CAA69226.1
AF157623 Genomic DNA Translation: AAD41525.1
CH471066 Genomic DNA Translation: EAW49312.1
CH471066 Genomic DNA Translation: EAW49313.1
AF097709 mRNA Translation: AAC97211.1

The Consensus CDS (CCDS) project

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CCDSi
CCDS7630.1

NCBI Reference Sequences

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RefSeqi
NP_002766.1, NM_002775.4

UniGene gene-oriented nucleotide sequence clusters

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UniGenei
Hs.501280

Genome annotation databases

Ensembl eukaryotic genome annotation project

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Ensembli
ENST00000368984; ENSP00000357980; ENSG00000166033

Database of genes from NCBI RefSeq genomes

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GeneIDi
5654

KEGG: Kyoto Encyclopedia of Genes and Genomes

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KEGGi
hsa:5654

UCSC genome browser

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UCSCi
uc001lgj.2 human

Keywords - Coding sequence diversityi

Polymorphism

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

<p>This subsection of the <a href="http://www.uniprot.org/manual/cross_references_section">Cross-references</a> section provides links to various web resources that are relevant for a specific protein.<p><a href='/help/web_resource' target='_top'>More...</a></p>Web resourcesi

Atlas of Genetics and Cytogenetics in Oncology and Haematology

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
Y07921 mRNA Translation: CAA69226.1
AF157623 Genomic DNA Translation: AAD41525.1
CH471066 Genomic DNA Translation: EAW49312.1
CH471066 Genomic DNA Translation: EAW49313.1
AF097709 mRNA Translation: AAC97211.1
CCDSiCCDS7630.1
RefSeqiNP_002766.1, NM_002775.4
UniGeneiHs.501280

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2JOANMR-A380-480[»]
2YTWNMR-A370-480[»]
3NUMX-ray2.75A158-480[»]
3NWUX-ray3.20A/B/C158-375[»]
3NZIX-ray2.75A158-480[»]
3TJNX-ray3.00A/B/D161-367[»]
3TJOX-ray2.30A/B/D161-370[»]
3TJQX-ray2.00A35-156[»]
ProteinModelPortaliQ92743
SMRiQ92743
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi111635, 20 interactors
DIPiDIP-33195N
IntActiQ92743, 10 interactors
MINTiQ92743
STRINGi9606.ENSP00000357980

Protein family/group databases

MEROPSiS01.277

PTM databases

iPTMnetiQ92743
PhosphoSitePlusiQ92743

Polymorphism and mutation databases

BioMutaiHTRA1
DMDMi18202620

Proteomic databases

EPDiQ92743
jPOSTiQ92743
PaxDbiQ92743
PeptideAtlasiQ92743
PRIDEiQ92743
ProteomicsDBi75437

Protocols and materials databases

The DNASU plasmid repository

More...
DNASUi
5654
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000368984; ENSP00000357980; ENSG00000166033
GeneIDi5654
KEGGihsa:5654
UCSCiuc001lgj.2 human

Organism-specific databases

Comparative Toxicogenomics Database

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CTDi
5654
DisGeNETi5654
EuPathDBiHostDB:ENSG00000166033.11

GeneCards: human genes, protein and diseases

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GeneCardsi
HTRA1
GeneReviewsiHTRA1
HGNCiHGNC:9476 HTRA1
HPAiHPA036655
MalaCardsiHTRA1
MIMi600142 phenotype
602194 gene
610149 phenotype
616779 phenotype
neXtProtiNX_Q92743
OpenTargetsiENSG00000166033
Orphaneti199354 CARASIL
482077 HTRA1-related autosomal dominant cerebral small vessel disease
3148 Malignant peripheral nerve sheath tumor
279 NON RARE IN EUROPE: Age-related macular degeneration
PharmGKBiPA33829

GenAtlas: human gene database

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GenAtlasi
Search...

Phylogenomic databases

eggNOGiKOG1320 Eukaryota
COG0265 LUCA
GeneTreeiENSGT00940000156955
HOGENOMiHOG000223641
HOVERGENiHBG052044
InParanoidiQ92743
KOiK08784
OMAiPERCEPA
OrthoDBi630723at2759
PhylomeDBiQ92743
TreeFamiTF323480

Enzyme and pathway databases

BRENDAi3.4.21.108 2681
ReactomeiR-HSA-1474228 Degradation of the extracellular matrix

Miscellaneous databases

ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

More...
ChiTaRSi
HTRA1 human
EvolutionaryTraceiQ92743

The Gene Wiki collection of pages on human genes and proteins

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GeneWikii
HTRA1

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

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GenomeRNAii
5654

Protein Ontology

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PROi
PR:Q92743

The Stanford Online Universal Resource for Clones and ESTs

More...
SOURCEi
Search...

Gene expression databases

BgeeiENSG00000166033 Expressed in 234 organ(s), highest expression level in tendon
CleanExiHS_HTRA1
ExpressionAtlasiQ92743 baseline and differential
GenevisibleiQ92743 HS

Family and domain databases

InterProiView protein in InterPro
IPR009030 Growth_fac_rcpt_cys_sf
IPR000867 IGFBP-like
IPR002350 Kazal_dom
IPR036058 Kazal_dom_sf
IPR001478 PDZ
IPR036034 PDZ_sf
IPR009003 Peptidase_S1_PA
IPR001940 Peptidase_S1C
PfamiView protein in Pfam
PF00219 IGFBP, 1 hit
PF07648 Kazal_2, 1 hit
PRINTSiPR00834 PROTEASES2C
SMARTiView protein in SMART
SM00121 IB, 1 hit
SM00280 KAZAL, 1 hit
SM00228 PDZ, 1 hit
SUPFAMiSSF100895 SSF100895, 1 hit
SSF50156 SSF50156, 1 hit
SSF50494 SSF50494, 1 hit
SSF57184 SSF57184, 1 hit
PROSITEiView protein in PROSITE
PS51323 IGFBP_N_2, 1 hit
PS51465 KAZAL_2, 1 hit
PS50106 PDZ, 1 hit

ProtoNet; Automatic hierarchical classification of proteins

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ProtoNeti
Search...

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This subsection of the ‘Entry information’ section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiHTRA1_HUMAN
<p>This subsection of the ‘Entry information’ section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called ‘Primary (citable) accession number’.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: Q92743
Secondary accession number(s): D3DRE4, Q9UNS5
<p>This subsection of the ‘Entry information’ section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification (‘Last modified’). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: September 26, 2001
Last sequence update: February 1, 1997
Last modified: January 16, 2019
This is version 177 of the entry and version 1 of the sequence. See complete history.
<p>This subsection of the ‘Entry information’ section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Peptidase families
    Classification of peptidase families and list of entries
  2. SIMILARITY comments
    Index of protein domains and families
  3. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  4. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  5. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  6. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  7. Human chromosome 10
    Human chromosome 10: entries, gene names and cross-references to MIM
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