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Protein

Peroxisomal leader peptide-processing protease

Gene

TYSND1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Peroxisomal protease that mediates both the removal of the leader peptide from proteins containing a PTS2 target sequence and processes several PTS1-containing proteins. Catalyzes the processing of PTS1-proteins involved in the peroxisomal beta-oxidation of fatty acids.1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei372Charge relay systemBy similarity1
Active sitei408Charge relay systemBy similarity1
Active sitei481Charge relay systemBy similarity1

GO - Molecular functioni

  • protease binding Source: UniProtKB
  • serine-type endopeptidase activity Source: UniProtKB

GO - Biological processi

  • protein homooligomerization Source: UniProtKB
  • protein processing Source: UniProtKB
  • proteolysis Source: UniProtKB
  • regulation of fatty acid beta-oxidation Source: UniProtKB
Complete GO annotation...

Keywords - Molecular functioni

Hydrolase, Protease, Serine protease

Protein family/group databases

MEROPSiS01.286.

Names & Taxonomyi

Protein namesi
Recommended name:
Peroxisomal leader peptide-processing protease (EC:3.4.21.-)
Alternative name(s):
Trypsin domain-containing protein 1
Cleaved into the following 2 chains:
Gene namesi
Name:TYSND1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 10

Organism-specific databases

HGNCiHGNC:28531. TYSND1.

Subcellular locationi

  • Peroxisome 1 Publication

GO - Cellular componenti

  • membrane Source: UniProtKB
  • peroxisome Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Peroxisome

Pathology & Biotechi

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Mutagenesisi481S → A: Abrogates the self-cleaving activity of TYSND1. 1 Publication1

Organism-specific databases

DisGeNETi219743.
OpenTargetsiENSG00000156521.
PharmGKBiPA134968651.

Polymorphism and mutation databases

DMDMi146325807.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00002861241 – 566Peroxisomal leader peptide-processing proteaseAdd BLAST566
ChainiPRO_00002861251 – 110Peroxisomal leader peptide-processing protease, 15 kDa formAdd BLAST110
ChainiPRO_0000286126111 – 566Peroxisomal leader peptide-processing protease, 45 kDa formAdd BLAST456

Post-translational modificationi

Self-cleavage gives rise to an N-terminal 15-kDa fragment and C-terminal 45-kDa fragment upon import into the peroxisomes. The full-lengh TYSND1 is the active the proteolytic processing of PTS1- and PTS2-proteins and in self-cleavage, and intermolecular self-cleavage of TYSND1 down-regulates its protease activity.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sitei110 – 111CleavageBy similarity2

Proteomic databases

EPDiQ2T9J0.
MaxQBiQ2T9J0.
PaxDbiQ2T9J0.
PeptideAtlasiQ2T9J0.
PRIDEiQ2T9J0.

PTM databases

iPTMnetiQ2T9J0.
PhosphoSitePlusiQ2T9J0.

Expressioni

Gene expression databases

BgeeiENSG00000156521.
CleanExiHS_TYSND1.
GenevisibleiQ2T9J0. HS.

Organism-specific databases

HPAiHPA030304.

Interactioni

Subunit structurei

Homodimer. Forms a heterodimer with the C-terminal cleavage product (45 kDa form). Forms a heterodimer with the N-terminal cleavage product (15 kDa form). Interacts with PEX5. Interacts with LONP2.1 Publication

GO - Molecular functioni

  • protease binding Source: UniProtKB

Protein-protein interaction databases

BioGridi128571. 11 interactors.
IntActiQ2T9J0. 12 interactors.
STRINGi9606.ENSP00000287078.

Structurei

3D structure databases

ProteinModelPortaliQ2T9J0.
SMRiQ2T9J0.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni319 – 531Serine proteaseAdd BLAST213

Sequence similaritiesi

Belongs to the peptidase S1B family.Curated

Phylogenomic databases

eggNOGiKOG1320. Eukaryota.
COG0265. LUCA.
GeneTreeiENSGT00390000014627.
HOGENOMiHOG000050236.
HOVERGENiHBG101259.
InParanoidiQ2T9J0.
OMAiCWKAREW.
OrthoDBiEOG091G06CU.
PhylomeDBiQ2T9J0.
TreeFamiTF331254.

Family and domain databases

InterProiIPR017345. Pept_S1A_Tysnd1.
IPR009003. Peptidase_S1_PA.
[Graphical view]
PIRSFiPIRSF037989. Peptidase_S1B_Tysnd1. 1 hit.
SUPFAMiSSF50494. SSF50494. 2 hits.

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q2T9J0-1) [UniParc]FASTAAdd to basket

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.

« Hide

        10         20         30         40         50
MRRQWGSAMR AAEQAGCMVS ASRAGQPEAG PWSCSGVILS RSPGLVLCHG
60 70 80 90 100
GIFVPFLRAG SEVLTAAGAV FLPGDSCRDD LRLHVQWAPT AAGPGGGAER
110 120 130 140 150
GRPGLCTPQC ASLEPGPPAP SRGRPLQPRL PAELLLLLSC PAFWAHFARL
160 170 180 190 200
FGDEAAEQWR FSSAARDDEV SEDEEADQLR ALGWFALLGV RLGQEEVEEE
210 220 230 240 250
RGPAMAVSPL GAVPKGAPLL VCGSPFGAFC PDIFLNTLSC GVLSNVAGPL
260 270 280 290 300
LLTDARCLPG TEGGGVFTAR PAGALVALVV APLCWKAGEW VGFTLLCAAA
310 320 330 340 350
PLFRAARDAL HRLPHSTAAL AALLPPEVGV PWGLPLRDSG PLWAAAAVLV
360 370 380 390 400
ECGTVWGSGV AVAPRLVVTC RHVSPREAAR VLVRSTTPKS VAIWGRVVFA
410 420 430 440 450
TQETCPYDIA VVSLEEDLDD VPIPVPAEHF HEGEAVSVVG FGVFGQSCGP
460 470 480 490 500
SVTSGILSAV VQVNGTPVML QTTCAVHSGS SGGPLFSNHS GNLLGIITSN
510 520 530 540 550
TRDNNTGATY PHLNFSIPIT VLQPALQQYS QTQDLGGLRE LDRAAEPVRV
560
VWRLQRPLAE APRSKL
Length:566
Mass (Da):59,309
Last modified:May 1, 2007 - v3
Checksum:i530B895B8698F2E6
GO
Isoform 2 (identifier: Q2T9J0-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     390-398: SVAIWGRVV → HNHQQHPGQ
     399-566: Missing.

Note: No experimental confirmation available.
Show »
Length:398
Mass (Da):41,573
Checksum:iE7AC4EFD235D465F
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti203P → L in AAH30242 (PubMed:15489334).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_05975865T → A.1 PublicationCorresponds to variant rs4746970dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_025000390 – 398SVAIWGRVV → HNHQQHPGQ in isoform 2. 1 Publication9
Alternative sequenceiVSP_025001399 – 566Missing in isoform 2. 1 PublicationAdd BLAST168

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AL731540 Genomic DNA. Translation: CAI13685.1.
AL731540 Genomic DNA. Translation: CAI13686.1.
BC016840 mRNA. Translation: AAH16840.1.
BC030242 mRNA. Translation: AAH30242.1.
BC111501 mRNA. Translation: AAI11502.2.
CCDSiCCDS31213.1. [Q2T9J0-1]
CCDS31214.1. [Q2T9J0-2]
RefSeqiNP_001035363.1. NM_001040273.2. [Q2T9J0-2]
NP_775826.2. NM_173555.3. [Q2T9J0-1]
UniGeneiHs.533655.

Genome annotation databases

EnsembliENST00000287078; ENSP00000287078; ENSG00000156521. [Q2T9J0-1]
ENST00000335494; ENSP00000335673; ENSG00000156521. [Q2T9J0-2]
GeneIDi219743.
KEGGihsa:219743.
UCSCiuc001jqr.5. human. [Q2T9J0-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AL731540 Genomic DNA. Translation: CAI13685.1.
AL731540 Genomic DNA. Translation: CAI13686.1.
BC016840 mRNA. Translation: AAH16840.1.
BC030242 mRNA. Translation: AAH30242.1.
BC111501 mRNA. Translation: AAI11502.2.
CCDSiCCDS31213.1. [Q2T9J0-1]
CCDS31214.1. [Q2T9J0-2]
RefSeqiNP_001035363.1. NM_001040273.2. [Q2T9J0-2]
NP_775826.2. NM_173555.3. [Q2T9J0-1]
UniGeneiHs.533655.

3D structure databases

ProteinModelPortaliQ2T9J0.
SMRiQ2T9J0.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi128571. 11 interactors.
IntActiQ2T9J0. 12 interactors.
STRINGi9606.ENSP00000287078.

Protein family/group databases

MEROPSiS01.286.

PTM databases

iPTMnetiQ2T9J0.
PhosphoSitePlusiQ2T9J0.

Polymorphism and mutation databases

DMDMi146325807.

Proteomic databases

EPDiQ2T9J0.
MaxQBiQ2T9J0.
PaxDbiQ2T9J0.
PeptideAtlasiQ2T9J0.
PRIDEiQ2T9J0.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000287078; ENSP00000287078; ENSG00000156521. [Q2T9J0-1]
ENST00000335494; ENSP00000335673; ENSG00000156521. [Q2T9J0-2]
GeneIDi219743.
KEGGihsa:219743.
UCSCiuc001jqr.5. human. [Q2T9J0-1]

Organism-specific databases

CTDi219743.
DisGeNETi219743.
GeneCardsiTYSND1.
HGNCiHGNC:28531. TYSND1.
HPAiHPA030304.
MIMi611017. gene.
neXtProtiNX_Q2T9J0.
OpenTargetsiENSG00000156521.
PharmGKBiPA134968651.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG1320. Eukaryota.
COG0265. LUCA.
GeneTreeiENSGT00390000014627.
HOGENOMiHOG000050236.
HOVERGENiHBG101259.
InParanoidiQ2T9J0.
OMAiCWKAREW.
OrthoDBiEOG091G06CU.
PhylomeDBiQ2T9J0.
TreeFamiTF331254.

Miscellaneous databases

ChiTaRSiTYSND1. human.
GenomeRNAii219743.
PROiQ2T9J0.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000156521.
CleanExiHS_TYSND1.
GenevisibleiQ2T9J0. HS.

Family and domain databases

InterProiIPR017345. Pept_S1A_Tysnd1.
IPR009003. Peptidase_S1_PA.
[Graphical view]
PIRSFiPIRSF037989. Peptidase_S1B_Tysnd1. 1 hit.
SUPFAMiSSF50494. SSF50494. 2 hits.
ProtoNetiSearch...

Entry informationi

Entry nameiTYSD1_HUMAN
AccessioniPrimary (citable) accession number: Q2T9J0
Secondary accession number(s): Q5SQT4
, Q5SQU1, Q8N6H2, Q96AR5
Entry historyi
Integrated into UniProtKB/Swiss-Prot: May 1, 2007
Last sequence update: May 1, 2007
Last modified: November 2, 2016
This is version 111 of the entry and version 3 of the sequence. [Complete history]
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.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

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

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.