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The preproprotein encoded by this gene, a member of the peptidase C1 family, is a lysosomal cysteine proteinase that participates in the degradation of antigenic proteins to peptides for presentation on MHC class II molecules. The mature protein cleaves the invariant chain of MHC class II molecules in endolysosomal compartments and enables the formation of antigen-MHC class II complexes and the proper display of extracellular antigenic peptides by MHC-II. The mature protein also functions as an elastase over a broad pH range. When secreted from cells, this protein can remodel components of the extracellular matrix such as elastin, collagen, and fibronectin. This gene is implicated in the pathology of many inflammatory and autoimmune diseases and, given its elastase activity, plays a significant role in some pulmonary diseases. Alternatively spliced transcript variants encoding distinct isoforms have been found for this gene. [provided by RefSeq, May 2020]
CTSS (Cathepsin S) is a Protein Coding gene. Diseases associated with CTSS include Dacryoadenitis and Cerebellopontine Angle Tumor. Among its related pathways are Bacterial infections in CF airways and Degradation of the extracellular matrix. Gene Ontology (GO) annotations related to this gene include peptidase activity and cysteine-type peptidase activity. An important paralog of this gene is CTSK.
GO ID | Qualified GO term | Evidence | PubMed IDs |
---|---|---|---|
GO:0001968 | fibronectin binding | IPI | 22952693 |
GO:0004197 | cysteine-type endopeptidase activity | IDA,TAS | -- |
GO:0005518 | collagen binding | IDA | 22952693 |
GO:0008233 | peptidase activity | IEA | -- |
GO:0008234 | cysteine-type peptidase activity | IEA | -- |
GO ID | Qualified GO term | Evidence | PubMed IDs |
---|---|---|---|
GO:0005576 | extracellular region | TAS | -- |
GO:0005615 | extracellular space | IBA,IDA | 9524075 |
GO:0005764 | lysosome | IBA,IDA | 15196205 |
GO:0005770 | late endosome | IDA | 22365146 |
GO:0031410 | cytoplasmic vesicle | IEA | -- |
SuperPathway | Contained pathways | ||
---|---|---|---|
1 | Innate Immune System |
.61
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2 | Class I MHC mediated antigen processing and presentation | ||
3 | Antigen processing-Cross presentation | ||
4 | Collagen chain trimerization | ||
5 | Degradation of the extracellular matrix |
GO ID | Qualified GO term | Evidence | PubMed IDs |
---|---|---|---|
GO:0002224 | toll-like receptor signaling pathway | TAS | -- |
GO:0002250 | adaptive immune response | IEP | 15196205 |
GO:0006508 | proteolysis | IEA,IDA | 12788072 |
GO:0006955 | immune response | TAS,IBA | 21873635 |
GO:0010447 | response to acidic pH | IDA | 22864553 |
Name | Status | Disease Links | Group | Role | Mechanism of Action | Clinical Trials |
---|---|---|---|---|---|---|
fostamatinib | Approved, Investigational | Pharma | Target, inhibitor | Kinase Inhibitors | 0 | |
(1R)-2-[(CYANOMETHYL)AMINO]-1-({[2-(DIFLUOROMETHOXY)BENZYL]SULFONYL}METHYL)-2-OXOETHYL MORPHOLINE-4-CARBOXYLATE | Experimental | Pharma | Target | 0 | ||
2-[(2',3',4'-TRIFLUOROBIPHENYL-2-YL)OXY]ETHANOL | Experimental | Pharma | Target | 0 | ||
Morpholine-4-Carboxylic Acid (1-(3-Benzenesulfonyl-1-Phenethylallylcarbamoyl)-3-Methylbutyl)-Amide | Experimental | Pharma | Target | 0 | ||
Morpholine-4-Carboxylic Acid [1-(2-Benzylsulfanyl-1-Formyl-Ethylcarbamoyl)-2-Phenyl-Ethyl]-Amide | Experimental | Pharma | Target | 0 |
Compound | Action | Cas Number |
---|---|---|
Cathepsin Inhibitor 1 | Cathepsin inhibitor | 225120-65-0 |
Cathepsin S inhibitor | Blocks MHCII antigen presentation | 1373215-15-6 |
E-64 | Cysteine protease inhibitor,irriversible | 66701-25-5 |
ExUns: | 1a | · | 1b | ^ | 2 | ^ | 3 | ^ | 4a | · | 4b | · | 4c | ^ | 5 | ^ | 6 | ^ | 7 | ^ | 8 | ^ | 9a | · | 9b | · | 9c | ^ | 10 |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
SP1: | - | - | |||||||||||||||||||||||||||
SP2: | - | ||||||||||||||||||||||||||||
SP3: | |||||||||||||||||||||||||||||
SP4: | - | - | - | ||||||||||||||||||||||||||
SP5: | - |
This gene was present in the common ancestor of eukaryotes.
Organism | Taxonomy | Gene | Similarity | Type | Details |
---|---|---|---|---|---|
Chimpanzee (Pan troglodytes) |
Mammalia | CTSS 30 31 |
|
OneToOne | |
Dog (Canis familiaris) |
Mammalia | CTSS 30 31 |
|
OneToOne | |
Cow (Bos Taurus) |
Mammalia | CTSS 30 31 |
|
OneToOne | |
Rat (Rattus norvegicus) |
Mammalia | Ctss 30 |
|
||
Mouse (Mus musculus) |
Mammalia | Ctss 30 17 31 |
|
OneToOne | |
Oppossum (Monodelphis domestica) |
Mammalia | CTSS 31 |
|
OneToOne | |
Platypus (Ornithorhynchus anatinus) |
Mammalia | CTSS 31 |
|
OneToOne | |
Chicken (Gallus gallus) |
Aves | CTSS 30 31 |
|
OneToOne | |
Lizard (Anolis carolinensis) |
Reptilia | CTSS 31 |
|
OneToOne | |
Tropical Clawed Frog (Silurana tropicalis) |
Amphibia | ctss 30 |
|
||
African clawed frog (Xenopus laevis) |
Amphibia | ctss-prov 30 |
|
||
Zebrafish (Danio rerio) |
Actinopterygii | ctssb.1 30 31 |
|
OneToMany | |
ctssb.2 31 |
|
OneToMany | |||
-- 30 |
|
||||
Rainbow Trout (Oncorhynchus mykiss) |
Actinopterygii | Omy.8503 30 |
|
||
Fruit Fly (Drosophila melanogaster) |
Insecta | Cp1 32 |
|
|
|
CG4847 32 |
|
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|||
CG11459 32 |
|
|
|||
26-29kD-proteinase 32 |
|
|
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Worm (Caenorhabditis elegans) |
Secernentea | cpl-1 32 |
|
|
|
R09F10.1 32 |
|
|
|||
Thale Cress (Arabidopsis thaliana) |
eudicotyledons | AT4G11320 30 |
|
SNP ID | Clinical significance and condition | Chr 01 pos | Variation | AA Info | Type |
---|---|---|---|---|---|
rs140408537 | Benign: not provided | 150,747,785(-) |
C/A NM_004079.5(CTSS):c.888G>T (p.Val296=) |
SYNONYMOUS | |
rs16840587 | Benign: not provided | 150,751,772(-) |
A/T NM_004079.5(CTSS):c.627+9T>A |
INTRON | |
rs1059604 | - |
p.Ser161Thr |
|||
rs2230061 | - |
p.Arg113Trp |
Variant ID | Type | Subtype | PubMed ID |
---|---|---|---|
esv1299298 | CNV | deletion | 17803354 |
esv1542672 | CNV | deletion | 17803354 |
esv2717818 | CNV | deletion | 23290073 |
esv3587511 | CNV | loss | 21293372 |
nsv1006838 | CNV | gain | 25217958 |
nsv1008381 | CNV | gain | 25217958 |
nsv1076858 | CNV | deletion | 25765185 |
nsv1144404 | CNV | deletion | 24896259 |
nsv437169 | CNV | loss | 16327808 |
nsv952803 | CNV | deletion | 24416366 |
Disorder | Aliases | PubMed IDs |
---|---|---|
dacryoadenitis |
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cerebellopontine angle tumor |
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