Q76LX8: A disintegrin and metalloproteinase with thrombospondin motifs 13

General Information

Protein names	- A disintegrin and metalloproteinase with thrombospondin motifs 13 - ADAM-TS 13 - ADAM-TS13 - ADAMTS-13 - 3.4.24.87 {ECO:0000269\|PubMed:11535495} - von Willebrand factor-cleaving protease - vWF-CP - vWF-cleaving protease
Gene names	ADAMTS13
Organism	Homo sapiens
Protease Family	M12.241
Protease ID	M12.241
Chromosome location
UniProt ID	Q76LX8

7 N-termini

4 C-termini

8 Cleavages

1 Substrates

Sequence

Isoforms

- Isoform 2 of A disintegrin and metalloproteinase with thrombospondin motifs 13 - Isoform 3 of A disintegrin and metalloproteinase with thrombospondin motifs 13 - Isoform 4 of A disintegrin and metalloproteinase with thrombospondin motifs 13

Sequence View

        10         20         30         40         50         60
MHQRHPRARC PPLCVAGILA CGFLLGCWGP SHFQQSCLQA LEPQAVSSYL SPGAPLKGRP
        70         80         90        100        110        120
PSPGFQRQRQ RQRRAAGGIL HLELLVAVGP DVFQAHQEDT ERYVLTNLNI GAELLRDPSL
       130        140        150        160        170        180
GAQFRVHLVK MVILTEPEGA PNITANLTSS LLSVCGWSQT INPEDDTDPG HADLVLYITR
       190        200        210        220        230        240
FDLELPDGNR QVRGVTQLGG ACSPTWSCLI TEDTGFDLGV TIAHEIGHSF GLEHDGAPGS
       250        260        270        280        290        300
GCGPSGHVMA SDGAAPRAGL AWSPCSRRQL LSLLSAGRAR CVWDPPRPQP GSAGHPPDAQ
       310        320        330        340        350        360
PGLYYSANEQ CRVAFGPKAV ACTFAREHLD MCQALSCHTD PLDQSSCSRL LVPLLDGTEC
       370        380        390        400        410        420
GVEKWCSKGR CRSLVELTPI AAVHGRWSSW GPRSPCSRSC GGGVVTRRRQ CNNPRPAFGG
       430        440        450        460        470        480
RACVGADLQA EMCNTQACEK TQLEFMSQQC ARTDGQPLRS SPGGASFYHW GAAVPHSQGD
       490        500        510        520        530        540
ALCRHMCRAI GESFIMKRGD SFLDGTRCMP SGPREDGTLS LCVSGSCRTF GCDGRMDSQQ
       550        560        570        580        590        600
VWDRCQVCGG DNSTCSPRKG SFTAGRAREY VTFLTVTPNL TSVYIANHRP LFTHLAVRIG
       610        620        630        640        650        660
GRYVVAGKMS ISPNTTYPSL LEDGRVEYRV ALTEDRLPRL EEIRIWGPLQ EDADIQVYRR
       670        680        690        700        710        720
YGEEYGNLTR PDITFTYFQP KPRQAWVWAA VRGPCSVSCG AGLRWVNYSC LDQARKELVE
       730        740        750        760        770        780
TVQCQGSQQP PAWPEACVLE PCPPYWAVGD FGPCSASCGG GLRERPVRCV EAQGSLLKTL
       790        800        810        820        830        840
PPARCRAGAQ QPAVALETCN PQPCPARWEV SEPSSCTSAG GAGLALENET CVPGADGLEA
       850        860        870        880        890        900
PVTEGPGSVD EKLPAPEPCV GMSCPPGWGH LDATSAGEKA PSPWGSIRTG AQAAHVWTPA
       910        920        930        940        950        960
AGSCSVSCGR GLMELRFLCM DSALRVPVQE ELCGLASKPG SRREVCQAVP CPARWQYKLA
       970        980        990       1000       1010       1020
ACSVSCGRGV VRRILYCARA HGEDDGEEIL LDTQCQGLPR PEPQEACSLE PCPPRWKVMS
      1030       1040       1050       1060       1070       1080
LGPCSASCGL GTARRSVACV QLDQGQDVEV DEAACAALVR PEASVPCLIA DCTYRWHVGT
      1090       1100       1110       1120       1130       1140
WMECSVSCGD GIQRRRDTCL GPQAQAPVPA DFCQHLPKPV TVRGCWAGPC VGQGTPSLVP
      1150       1160       1170       1180       1190       1200
HEEAAAPGRT TATPAGASLE WSQARGLLFS PAPQPRRLLP GPQENSVQSS ACGRQHLEPT
      1210       1220       1230       1240       1250       1260
GTIDMRGPGQ ADCAVAIGRP LGEVVTLRVL ESSLNCSAGD MLLLWGRLTW RKMCRKLLDM
      1270       1280       1290       1300       1310       1320
TFSSKTNTLV VRQRCGRPGG GVLLRYGSQL APETFYRECD MQLFGPWGEI VSPSLSPATS
      1330       1340       1350       1360       1370       1380
NAGGCRLFIN VAPHARIAIH ALATNMGAGT EGANASYILI RDTHSLRTTA FHGQQVLYWE
      1390       1400       1410       1420
SESSQAEMEF SEGFLKAQAS LRGQYWTLQS WVPEMQDPQS WKGKEGT         10         20         30         40         50         60
MHQRHPRARC PPLCVAGILA CGFLLGCWGP SHFQQSCLQA LEPQAVSSYL SPGAPLKGRP
        70         80         90        100        110        120
PSPGFQRQRQ RQRRAAGGIL HLELLVAVGP DVFQAHQEDT ERYVLTNLNI GAELLRDPSL
       130        140        150        160        170        180
GAQFRVHLVK MVILTEPEGA PNITANLTSS LLSVCGWSQT INPEDDTDPG HADLVLYITR
       190        200        210        220        230        240
FDLELPDGNR QVRGVTQLGG ACSPTWSCLI TEDTGFDLGV TIAHEIGHSF GLEHDGAPGS
       250        260        270        280        290        300
GCGPSGHVMA SDGAAPRAGL AWSPCSRRQL LSLLSAGRAR CVWDPPRPQP GSAGHPPDAQ
       310        320        330        340        350        360
PGLYYSANEQ CRVAFGPKAV ACTFAREHLD MCQALSCHTD PLDQSSCSRL LVPLLDGTEC
       370        380        390        400        410        420
GVEKWCSKGR CRSLVELTPI AAVHGRWSSW GPRSPCSRSC GGGVVTRRRQ CNNPRPAFGG
       430        440        450        460        470        480
RACVGADLQA EMCNTQACEK TQLEFMSQQC ARTDGQPLRS SPGGASFYHW GAAVPHSQGD
       490        500        510        520        530        540
ALCRHMCRAI GESFIMKRGD SFLDGTRCMP SGPREDGTLS LCVSGSCRTF GCDGRMDSQQ
       550        560        570        580        590        600
VWDRCQVCGG DNSTCSPRKG SFTAGRAREY VTFLTVTPNL TSVYIANHRP LFTHLAVRIG
       610        620        630        640        650        660
GRYVVAGKMS ISPNTTYPSL LEDGRVEYRV ALTEDRLPRL EEIRIWGPLQ EDADIQVYRR
       670        680        690        700        710        720
YGEEYGNLTR PDITFTYFQP KPRQAWVWAA VRGPCSVSCG AGLRWVNYSC LDQARKELVE
       730        740        750        760        770        780
TVQCQGSQQP PAWPEACVLE PCPPYWAVGD FGPCSASCGG GLRERPVRCV EAQGSLLKTL
       790        800        810        820        830        840
PPARCRAGAQ QPAVALETCN PQPCPARWEV SEPSSCTSAG GAGLALENET CVPGADGLEA
       850        860        870        880        890        900
PVTEGPGSVD EKLPAPEPCV GMSCPPGWGH LDATSAGEKA PSPWGSIRTG AQAAHVWTPA
       910        920        930        940        950        960
AGSCSVSCGR GLMELRFLCM DSALRVPVQE ELCGLASKPG SRREVCQAVP CPARWQYKLA
       970        980        990       1000       1010       1020
ACSVSCGRGV VRRILYCARA HGEDDGEEIL LDTQCQGLPR PEPQEACSLE PCPPRWKVMS
      1030       1040       1050       1060       1070       1080
LGPCSASCGL GTARRSVACV QLDQGQDVEV DEAACAALVR PEASVPCLIA DCTYRWHVGT
      1090       1100       1110       1120       1130       1140
WMECSVSCGD GIQRRRDTCL GPQAQAPVPA DFCQHLPKPV TVRGCWAGPC VGQGTPSLVP
      1150       1160       1170       1180       1190       1200
HEEAAAPGRT TATPAGASLE WSQARGLLFS PAPQPRRLLP GPQENSVQSS ACGRQHLEPT
      1210       1220       1230       1240       1250       1260
GTIDMRGPGQ ADCAVAIGRP LGEVVTLRVL ESSLNCSAGD MLLLWGRLTW RKMCRKLLDM
      1270       1280       1290       1300       1310       1320
TFSSKTNTLV VRQRCGRPGG GVLLRYGSQL APETFYRECD MQLFGPWGEI VSPSLSPATS
      1330       1340       1350       1360       1370       1380
NAGGCRLFIN VAPHARIAIH ALATNMGAGT EGANASYILI RDTHSLRTTA FHGQQVLYWE
      1390       1400       1410       1420
SESSQAEMEF SEGFLKAQAS LRGQYWTLQS WVPEMQDPQS WKGKEGT         10         20         30         40         50         60
MHQRHPRARC PPLCVAGILA CGFLLGCWGP SHFQQSCLQA LEPQAVSSYL SPGAPLKGRP
        70         80         90        100        110        120
PSPGFQRQRQ RQRRAAGGIL HLELLVAVGP DVFQAHQEDT ERYVLTNLNI GAELLRDPSL
       130        140        150        160        170        180
GAQFRVHLVK MVILTEPEGA PNITANLTSS LLSVCGWSQT INPEDDTDPG HADLVLYITR
       190        200        210        220        230        240
FDLELPDGNR QVRGVTQLGG ACSPTWSCLI TEDTGFDLGV TIAHEIGHSF GLEHDGAPGS
       250        260        270        280        290        300
GCGPSGHVMA SDGAAPRAGL AWSPCSRRQL LSLLSAGRAR CVWDPPRPQP GSAGHPPDAQ
       310        320        330        340        350        360
PGLYYSANEQ CRVAFGPKAV ACTFAREHLD MCQALSCHTD PLDQSSCSRL LVPLLDGTEC
       370        380        390        400        410        420
GVEKWCSKGR CRSLVELTPI AAVHGRWSSW GPRSPCSRSC GGGVVTRRRQ CNNPRPAFGG
       430        440        450        460        470        480
RACVGADLQA EMCNTQACEK TQLEFMSQQC ARTDGQPLRS SPGGASFYHW GAAVPHSQGD
       490        500        510        520        530        540
ALCRHMCRAI GESFIMKRGD SFLDGTRCMP SGPREDGTLS LCVSGSCRTF GCDGRMDSQQ
       550        560        570        580        590        600
VWDRCQVCGG DNSTCSPRKG SFTAGRAREY VTFLTVTPNL TSVYIANHRP LFTHLAVRIG
       610        620        630        640        650        660
GRYVVAGKMS ISPNTTYPSL LEDGRVEYRV ALTEDRLPRL EEIRIWGPLQ EDADIQVYRR
       670        680        690        700        710        720
YGEEYGNLTR PDITFTYFQP KPRQAWVWAA VRGPCSVSCG AGLRWVNYSC LDQARKELVE
       730        740        750        760        770        780
TVQCQGSQQP PAWPEACVLE PCPPYWAVGD FGPCSASCGG GLRERPVRCV EAQGSLLKTL
       790        800        810        820        830        840
PPARCRAGAQ QPAVALETCN PQPCPARWEV SEPSSCTSAG GAGLALENET CVPGADGLEA
       850        860        870        880        890        900
PVTEGPGSVD EKLPAPEPCV GMSCPPGWGH LDATSAGEKA PSPWGSIRTG AQAAHVWTPA
       910        920        930        940        950        960
AGSCSVSCGR GLMELRFLCM DSALRVPVQE ELCGLASKPG SRREVCQAVP CPARWQYKLA
       970        980        990       1000       1010       1020
ACSVSCGRGV VRRILYCARA HGEDDGEEIL LDTQCQGLPR PEPQEACSLE PCPPRWKVMS
      1030       1040       1050       1060       1070       1080
LGPCSASCGL GTARRSVACV QLDQGQDVEV DEAACAALVR PEASVPCLIA DCTYRWHVGT
      1090       1100       1110       1120       1130       1140
WMECSVSCGD GIQRRRDTCL GPQAQAPVPA DFCQHLPKPV TVRGCWAGPC VGQGTPSLVP
      1150       1160       1170       1180       1190       1200
HEEAAAPGRT TATPAGASLE WSQARGLLFS PAPQPRRLLP GPQENSVQSS ACGRQHLEPT
      1210       1220       1230       1240       1250       1260
GTIDMRGPGQ ADCAVAIGRP LGEVVTLRVL ESSLNCSAGD MLLLWGRLTW RKMCRKLLDM
      1270       1280       1290       1300       1310       1320
TFSSKTNTLV VRQRCGRPGG GVLLRYGSQL APETFYRECD MQLFGPWGEI VSPSLSPATS
      1330       1340       1350       1360       1370       1380
NAGGCRLFIN VAPHARIAIH ALATNMGAGT EGANASYILI RDTHSLRTTA FHGQQVLYWE
      1390       1400       1410       1420
SESSQAEMEF SEGFLKAQAS LRGQYWTLQS WVPEMQDPQS WKGKEGT

Filter Information:

(REFRESH)

Directness:

Physiological Relevance:

Evidence Codes:

Methodology:

Perturbation of System:

Biological System:

Protease Assignment Confidence:

Evidence Names:

Database:

Lab:

Protein Neighborhood

Domains & Features

7 N-termini - 4 C-termini - 8 Cleavages - 1 Substrates

N-termini

Name	Sequence	Position	Evidence type	Method	Source (database)	Evidence name	Publications (PMID)
Q76LX8-1-unknown	MHQRHP...	1	inferred from isoform by sequence similarity	unknown	TopFIND	inferred from TNt67971
Q76LX8-1-unknown	MHQRHP...	1	inferred from isoform by sequence similarity	unknown	TopFIND	inferred from TNt67972
Q76LX8-30-unknown	PSHFQQ...	30	inferred from electronic annotation	electronic annotation	UniProtKB	inferred from uniprot
Q76LX8-30-unknown	PSHFQQ...	30	inferred from isoform by sequence similarity	unknown	TopFIND	inferred from TNt114621
Q76LX8-30-unknown	PSHFQQ...	30	inferred from isoform by sequence similarity	unknown	TopFIND	inferred from TNt114622
Q76LX8-75-unknown	AAGGIL...	75	inferred from electronic annotation	electronic annotation	UniProtKB	inferred from uniprot
Q76LX8-75-unknown	AAGGIL...	75	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC26646
Q76LX8-75-unknown	AAGGIL...	75	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC26916
Q76LX8-75-unknown	AAGGIL...	75	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27214
Q76LX8-75-unknown	AAGGIL...	75	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC9808
Q76LX8-75-unknown	AAGGIL...	75	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27064
Q76LX8-75-unknown	AAGGIL...	75	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27361
Q76LX8-75-unknown	AAGGIL...	75	inferred from isoform by sequence similarity	unknown	TopFIND	inferred from TNt115788
Q76LX8-75-unknown	AAGGIL...	75	inferred from isoform by sequence similarity	unknown	TopFIND	inferred from TNt115789
Q76LX8-258-unknown	AGLAWS...	258	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC31810
Q76LX8-258-unknown	AGLAWS...	258	inferred from isoform by sequence similarity	unknown	TopFIND	inferred from TNt148738
Q76LX8-1166-unknown	GLLFSP...	1166		unknown		Wildes D. et al: Sampling the N-terminal proteome of human blood	20173099
Q76LX8-1177-unknown	RLLPGP...	1177	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC31813
Q76LX8-1205-unknown	MRGPGQ...	1205	inferred from electronic annotation	unknown	Ensembl	inferred from ensembl protein ENSP00000454450
Q76LX8-1205-unknown	MRGPGQ...	1205	inferred from electronic annotation	unknown	Ensembl	inferred from ensembl protein ENSP00000360978
Q76LX8-1205-unknown	MRGPGQ...	1205	inferred from isoform by sequence similarity	unknown	TopFIND	inferred from TNt200610
Q76LX8-1205-unknown	MRGPGQ...	1205	inferred from isoform by sequence similarity	unknown	TopFIND	inferred from TNt200611

C-termini

Sequence	Position	Evidence type	Method	Source (database)	Evidence name
...QRQRRA	74	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC26646
...QRQRRA	74	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC26916
...QRQRRA	74	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27214
...QRQRRA	74	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC9808
...QRQRRA	74	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27064
...QRQRRA	74	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC27361
...QRQRRA	74	inferred from isoform by sequence similarity	unknown	TopFIND	inferred from TCt124093
...QRQRRA	74	inferred from isoform by sequence similarity	unknown	TopFIND	inferred from TCt124094
...GAAPRA	257	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC31810
...GAAPRA	257	inferred from isoform by sequence similarity	unknown	TopFIND	inferred from TCt132113
...GAAPRA	257	inferred from isoform by sequence similarity	unknown	TopFIND	inferred from TCt132114
...APQPRR	1176	inferred from cleavage	unknown	TopFIND	Inferred from cleavage TC31813
...GKEGT	1427	inferred from electronic annotation	electronic annotation	UniProtKB	inferred from uniprot
...GKEGT	1427	inferred from isoform by sequence similarity	unknown	TopFIND	inferred from TCt63590
...GKEGT	1427	inferred from isoform by sequence similarity	unknown	TopFIND	inferred from TCt63589

Cleavages

Protease	Position	Sequence	Evidence type	Method	Source (database)	Source (Lab)	Evidence name	Publications (PMIDs)
NEC2_HUMAN	74	QRRA.\|.AAGG	inferred from experiment	unknown	MEROPS	Strongin AY	Remacle AG et al.:Substrate cleavage analysis of ... (S08.071)	18505722,
PCSK4_HUMAN	74	QRRA.\|.AAGG	inferred from experiment	unknown	MEROPS	Strongin AY	Remacle AG et al.:Substrate cleavage analysis of ... (S08.071)	18505722,
PCSK6_HUMAN	74	QRRA.\|.AAGG	inferred from experiment	unknown	MEROPS	Strongin AY	Remacle AG et al.:Substrate cleavage analysis of ... (S08.071)	18505722,
FURIN_HUMAN	74	QRRA.\|.AAGG	inferred from experiment	unknown	MEROPS	Strongin AY	Remacle AG et al.:Substrate cleavage analysis of ... (S08.071)	18505722,
PCSK5_HUMAN	74	QRRA.\|.AAGG	inferred from experiment	unknown	MEROPS	Strongin AY	Remacle AG et al.:Substrate cleavage analysis of ... (S08.071)	18505722,
PCSK7_HUMAN	74	QRRA.\|.AAGG	inferred from experiment	unknown	MEROPS	Strongin AY	Remacle AG et al.:Substrate cleavage analysis of ... (S08.071)	18505722,
THRB_HUMAN	257	APRA.\|.AGLA	inferred from experiment	unknown	MEROPS	Crawley JT	Lam JK et al.:Further characterization of ADA... (S01.217)	17355572,
THRB_HUMAN	1176	QPRR.\|.RLLP	inferred from experiment	unknown	MEROPS	Crawley JT	Lam JK et al.:Further characterization of ADA... (S01.217)	17355572,

Substrates

Substrate	Position	Sequence	Evidence type	Method	Source (database)	Source (Lab)	Evidence name	Publications (PMIDs)
VWF_HUMAN	1605	LVYM.\|.MVTG	inferred from experiment	unknown	MEROPS	Nozaki C	Soejima K et al.:A novel human metalloprotease s... (M12.241)	11574066,