Skip Header

You are using a version of Internet Explorer that may not display all features of this website. Please upgrade to a modern browser.
Contribute Send feedback
Read comments (?) or add your own

Q8K1T0 (TMPS3_MOUSE) Reviewed, UniProtKB/Swiss-Prot

Last modified July 9, 2014. Version 102. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (3) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Alt products·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Transmembrane protease serine 3

EC=3.4.21.-
Gene names
Name:Tmprss3
OrganismMus musculus (Mouse) [Reference proteome]
Taxonomic identifier10090 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus

Protein attributes

Sequence length453 AA.
Sequence statusComplete.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

Probable serine protease that play a role in hearing. Acts as a permissive factor for cochlear hair cells survival and activation at the onset of hearing and is required for saccular hair cell survival. Activates ENaC (in vitro). Ref.1 Ref.3

Subcellular location

Endoplasmic reticulum membrane; Single-pass type II membrane protein Ref.1 Ref.3.

Tissue specificity

Strongly expressed in liver, cochlea, brain, cerebellum, spleen, lung, and muscle and at a lower degree in retina, kidney, and heart. Expressed in the spiral ganglion, the cells supporting the organ of Corti and the stria vascularis. Isoform 2 is strongly expressed only in the cochlea with very faint expression in the cerebellum, spleen and muscle. Ref.3

Post-translational modification

Undergoes autoproteolytic activation.

Sequence similarities

Belongs to the peptidase S1 family.

Contains 1 LDL-receptor class A domain.

Contains 1 peptidase S1 domain.

Contains 1 SRCR domain.

Alternative products

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]
Isoform 1 (identifier: Q8K1T0-1)

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.
Isoform 2 (identifier: Q8K1T0-2)

Also known as: F;

The sequence of this isoform differs from the canonical sequence as follows:
     1-1: M → MAASEMVEVEPEPNIRGPEIVTM

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 453453Transmembrane protease serine 3
PRO_0000088691

Regions

Topological domain1 – 4848Cytoplasmic Potential
Transmembrane49 – 6921Helical; Signal-anchor for type II membrane protein; Potential
Topological domain70 – 453384Extracellular Potential
Domain72 – 10837LDL-receptor class A
Domain104 – 205102SRCR
Domain217 – 448232Peptidase S1

Sites

Active site2571Charge relay system By similarity
Active site3041Charge relay system By similarity
Active site4001Charge relay system Probable
Site216 – 2172Cleavage Potential

Amino acid modifications

Glycosylation2211N-linked (GlcNAc...) Potential
Disulfide bond73 ↔ 85 By similarity
Disulfide bond79 ↔ 98 By similarity
Disulfide bond92 ↔ 107 By similarity
Disulfide bond129 ↔ 194 By similarity
Disulfide bond142 ↔ 204 By similarity
Disulfide bond207 ↔ 324 By similarity
Disulfide bond242 ↔ 258 By similarity
Disulfide bond338 ↔ 406 By similarity
Disulfide bond369 ↔ 385 By similarity
Disulfide bond396 ↔ 424 By similarity

Natural variations

Alternative sequence11M → MAASEMVEVEPEPNIRGPEI VTM in isoform 2.
VSP_041581

Experimental info

Sequence conflict1171L → H in CAC83350. Ref.1
Sequence conflict2461I → V in CAD22137. Ref.1

Sequences

Sequence LengthMass (Da)Tools
Isoform 1 [UniParc].

Last modified July 27, 2011. Version 2.
Checksum: 1EE7ECD6CB3DD894

FASTA45349,506
        10         20         30         40         50         60 
MGENDPPAAE APFSFRSLFG LDDLKISPVA PDGDAVAAQI LSLLPLKFFP IIVIGIIALI 

        70         80         90        100        110        120 
LALAIGLGIH FDCSGKYRCH SSFKCIELTA RCDGVSDCKN AEDEYRCVRV SGQRAALQVF 

       130        140        150        160        170        180 
TAAAWRTMCS DDWKSHYAKI ACAQLGFPSY VSSDHLRVDA LEEQFQGDFV SINHLLSDDK 

       190        200        210        220        230        240 
VTALHHSVYM REGCTSGHVV TLKCSACGTR TGYSPRIVGG NMSSLTQWPW QVSLQFQGYH 

       250        260        270        280        290        300 
LCGGSIITPL WIVTAAHCVY DLYHPKSWTV QVGLVSLMDS PVPSHLVEKI IYHSKYKPKR 

       310        320        330        340        350        360 
LGNDIALMKL SEPLTFDETI QPICLPNSEE NFPDGKLCWT SGWGATEDGG DASPVLNHAA 

       370        380        390        400        410        420 
VPLISNKICN HRDVYGGIIS PSMLCAGYLK GGVDSCQGDS GGPLVCQERR LWKLVGATSF 

       430        440        450 
GIGCAEVNKP GVYTRITSFL DWIHEQLERD LKT 

« Hide

Isoform 2 (F) [UniParc].

Checksum: 0214D1E550EC917B
Show »

FASTA47551,886

References

[1]"The transmembrane serine protease (TMPRSS3) mutated in deafness DFNB8/10 activates the epithelial sodium channel (ENaC) in vitro."
Guipponi M., Vuagniaux G., Wattenhofer M., Shibuya K., Vazquez M., Dougherty L., Scamuffa N., Guida E., Okui M., Rossier C., Hancock M., Buchet K., Reymond A., Hummler E., Marzella P.L., Kudoh J., Shimizu N., Scott H.S., Antonarakis S.E., Rossier B.C.
Hum. Mol. Genet. 11:2829-2836(2002) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA / MRNA], SUBCELLULAR LOCATION, FUNCTION IN ENAC CLEAVAGE.
[2]"Genomic organization of murine transmembrane proteinases."
Rao N.V., Rao G.N., Hoidal J.R.
Submitted (MAR-2003) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA].
[3]"Tmprss3, a transmembrane serine protease deficient in human DFNB8/10 deafness, is critical for cochlear hair cell survival at the onset of hearing."
Fasquelle L., Scott H.S., Lenoir M., Wang J., Rebillard G., Gaboyard S., Venteo S., Francois F., Mausset-Bonnefont A.L., Antonarakis S.E., Neidhart E., Chabbert C., Puel J.L., Guipponi M., Delprat B.
J. Biol. Chem. 286:17383-17397(2011) [PubMed] [Europe PMC] [Abstract]
Cited for: FUNCTION, SUBCELLULAR LOCATION, TISSUE SPECIFICITY, ALTERNATIVE SPLICING (ISOFORM 2).
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
AJ429216 Genomic DNA. Translation: CAD22137.1.
AJ300738 mRNA. Translation: CAC83350.1.
AY261383 Genomic DNA. Translation: AAO33581.1.
CCDSCCDS37547.1. [Q8K1T0-1]
CCDS50053.1. [Q8K1T0-2]
RefSeqNP_542765.2. NM_080727.2. [Q8K1T0-1]
UniGeneMm.214638.

3D structure databases

ProteinModelPortalQ8K1T0.
SMRQ8K1T0. Positions 108-448.
ModBaseSearch...
MobiDBSearch...

Protein family/group databases

MEROPSS01.079.

PTM databases

PhosphoSiteQ8K1T0.

Proteomic databases

PRIDEQ8K1T0.

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENSMUST00000024833; ENSMUSP00000024833; ENSMUSG00000024034. [Q8K1T0-1]
ENSMUST00000114549; ENSMUSP00000110196; ENSMUSG00000024034. [Q8K1T0-2]
GeneID140765.
KEGGmmu:140765.
UCSCuc008bup.2. mouse. [Q8K1T0-1]

Organism-specific databases

CTD64699.
MGIMGI:2155445. Tmprss3.

Phylogenomic databases

eggNOGCOG5640.
GeneTreeENSGT00730000110467.
HOGENOMHOG000251822.
HOVERGENHBG013304.
InParanoidQ812A6.
KOK09634.
OMAVITPLWI.
OrthoDBEOG75B84T.
TreeFamTF351678.

Gene expression databases

BgeeQ8K1T0.
CleanExMM_TMPRSS3.
GenevestigatorQ8K1T0.

Family and domain databases

Gene3D3.10.250.10. 1 hit.
4.10.400.10. 1 hit.
InterProIPR023415. LDLR_class-A_CS.
IPR002172. LDrepeatLR_classA_rpt.
IPR001254. Peptidase_S1.
IPR018114. Peptidase_S1_AS.
IPR001314. Peptidase_S1A.
IPR001190. SRCR.
IPR017448. SRCR-like_dom.
IPR009003. Trypsin-like_Pept_dom.
[Graphical view]
PfamPF00057. Ldl_recept_a. 1 hit.
PF00089. Trypsin. 1 hit.
[Graphical view]
PRINTSPR00722. CHYMOTRYPSIN.
SMARTSM00192. LDLa. 1 hit.
SM00202. SR. 1 hit.
SM00020. Tryp_SPc. 1 hit.
[Graphical view]
SUPFAMSSF50494. SSF50494. 1 hit.
SSF56487. SSF56487. 1 hit.
SSF57424. SSF57424. 1 hit.
PROSITEPS01209. LDLRA_1. 1 hit.
PS50068. LDLRA_2. 1 hit.
PS50287. SRCR_2. 1 hit.
PS50240. TRYPSIN_DOM. 1 hit.
PS00134. TRYPSIN_HIS. 1 hit.
PS00135. TRYPSIN_SER. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

NextBio369973.
PROQ8K1T0.
SOURCESearch...

Entry information

Entry nameTMPS3_MOUSE
AccessionPrimary (citable) accession number: Q8K1T0
Secondary accession number(s): Q812A6, Q8VDE0
Entry history
Integrated into UniProtKB/Swiss-Prot: May 30, 2003
Last sequence update: July 27, 2011
Last modified: July 9, 2014
This is version 102 of the entry and version 2 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Relevant documents

SIMILARITY comments

Index of protein domains and families

Peptidase families

Classification of peptidase families and list of entries

MGD cross-references

Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot