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Protein

Peroxisomal leader peptide-processing protease

Gene

TYSND1

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

  • identical protein binding Source: IntAct
  • protease binding Source: UniProtKB
  • serine-type endopeptidase activity Source: UniProtKB

GO - Biological processi

Keywordsi

Molecular functionHydrolase, Protease, Serine protease

Enzyme and pathway databases

ReactomeiR-HSA-9033241 Peroxisomal protein import
R-HSA-9033500 TYSND1 cleaves peroxisomal proteins

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

EuPathDBiHostDB:ENSG00000156521.13
HGNCiHGNC:28531 TYSND1
MIMi611017 gene
neXtProtiNX_Q2T9J0

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

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
ProteomicsDBi61444
61445 [Q2T9J0-2]

PTM databases

iPTMnetiQ2T9J0
PhosphoSitePlusiQ2T9J0

Expressioni

Gene expression databases

BgeeiENSG00000156521 Expressed in 145 organ(s), highest expression level in testis
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

Protein-protein interaction databases

BioGridi128571, 12 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
OMAiCHGGIFT
OrthoDBiEOG091G06CU
PhylomeDBiQ2T9J0
TreeFamiTF331254

Family and domain databases

InterProiView protein in InterPro
IPR017345 Pept_S1A_Tysnd1
IPR009003 Peptidase_S1_PA
IPR039245 TYSND1/DEG15
PANTHERiPTHR21004 PTHR21004, 1 hit
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 → A1 PublicationCorresponds to variant dbSNP:rs4746970Ensembl.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 No translation available.
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

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AL731540 Genomic DNA No translation available.
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, 12 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
ProteomicsDBi61444
61445 [Q2T9J0-2]

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
EuPathDBiHostDB:ENSG00000156521.13
GeneCardsiTYSND1
HGNCiHGNC:28531 TYSND1
HPAiHPA030304
MIMi611017 gene
neXtProtiNX_Q2T9J0
OpenTargetsiENSG00000156521
PharmGKBiPA134968651
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG1320 Eukaryota
COG0265 LUCA
GeneTreeiENSGT00390000014627
HOGENOMiHOG000050236
HOVERGENiHBG101259
InParanoidiQ2T9J0
OMAiCHGGIFT
OrthoDBiEOG091G06CU
PhylomeDBiQ2T9J0
TreeFamiTF331254

Enzyme and pathway databases

ReactomeiR-HSA-9033241 Peroxisomal protein import
R-HSA-9033500 TYSND1 cleaves peroxisomal proteins

Miscellaneous databases

ChiTaRSiTYSND1 human
GenomeRNAii219743
PROiPR:Q2T9J0
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000156521 Expressed in 145 organ(s), highest expression level in testis
CleanExiHS_TYSND1
GenevisibleiQ2T9J0 HS

Family and domain databases

InterProiView protein in InterPro
IPR017345 Pept_S1A_Tysnd1
IPR009003 Peptidase_S1_PA
IPR039245 TYSND1/DEG15
PANTHERiPTHR21004 PTHR21004, 1 hit
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 historyiIntegrated into UniProtKB/Swiss-Prot: May 1, 2007
Last sequence update: May 1, 2007
Last modified: November 7, 2018
This is version 123 of the entry and version 3 of the sequence. See 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. SIMILARITY comments
    Index of protein domains and families
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Peptidase families
    Classification of peptidase families and list of entries
  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. Human chromosome 10
    Human chromosome 10: entries, gene names and cross-references to MIM
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Main funding by: National Institutes of Health

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