Uniprot ID:Q76LX8	Protein name: A disintegrin and metalloproteinase with thrombospondin motifs 13	Gene : ADAMTS13 C9orf8 UNQ6102/PRO20085
Protein Family:	Squence Length : 1427

Sequnce	>Q76LX8 1428 MHQRHPRARCPPLCVAGILACGFLLGCWGPSHFQQSCLQALEPQAVSSYLSPGAPLKGRPPSPGFQRQRQRQRRAAGGILHLELLVAVGPDVFQAHQEDTERYVLTNLNIGAELLRDPSLGAQFRVHLVKMVILTEPEGAPNITANLTSSLLSVCGWSQTINPEDDTDPGHADLVLYITRFDLELPDGNRQVRGVTQLGGACSPTWSCLITEDTGFDLGVTIAHEIGHSFGLEHDGAPGSGCGPSGHVMASDGAAPRAGLAWSPCSRRQLLSLLSAGRARCVWDPPRPQPGSAGHPPDAQPGLYYSANEQCRVAFGPKAVACTFAREHLDMCQALSCHTDPLDQSSCSRLLVPLLDGTECGVEKWCSKGRCRSLVELTPIAAVHGRWSSWGPRSPCSRSCGGGVVTRRRQCNNPRPAFGGRACVGADLQAEMCNTQACEKTQLEFMSQQCARTDGQPLRSSPGGASFYHWGAAVPHSQGDALCRHMCRAIGESFIMKRGDSFLDGTRCMPSGPREDGTLSLCVSGSCRTFGCDGRMDSQQVWDRCQVCGGDNSTCSPRKGSFTAGRAREYVTFLTVTPNLTSVYIANHRPLFTHLAVRIGGRYVVAGKMSISPNTTYPSLLEDGRVEYRVALTEDRLPRLEEIRIWGPLQEDADIQVYRRYGEEYGNLTRPDITFTYFQPKPRQAWVWAAVRGPCSVSCGAGLRWVNYSCLDQARKELVETVQCQGSQQPPAWPEACVLEPCPPYWAVGDFGPCSASCGGGLRERPVRCVEAQGSLLKTLPPARCRAGAQQPAVALETCNPQPCPARWEVSEPSSCTSAGGAGLALENETCVPGADGLEAPVTEGPGSVDEKLPAPEPCVGMSCPPGWGHLDATSAGEKAPSPWGSIRTGAQAAHVWTPAAGSCSVSCGRGLMELRFLCMDSALRVPVQEELCGLASKPGSRREVCQAVPCPARWQYKLAACSVSCGRGVVRRILYCARAHGEDDGEEILLDTQCQGLPRPEPQEACSLEPCPPRWKVMSLGPCSASCGLGTARRSVACVQLDQGQDVEVDEAACAALVRPEASVPCLIADCTYRWHVGTWMECSVSCGDGIQRRRDTCLGPQAQAPVPADFCQHLPKPVTVRGCWAGPCVGQGTPSLVPHEEAAAPGRTTATPAGASLEWSQARGLLFSPAPQPRRLLPGPQENSVQSSACGRQHLEPTGTIDMRGPGQADCAVAIGRPLGEVVTLRVLESSLNCSAGDMLLLWGRLTWRKMCRKLLDMTFSSKTNTLVVRQRCGRPGGGVLLRYGSQLAPETFYRECDMQLFGPWGEIVSPSLSPATSNAGGCRLFINVAPHARIAIHALATNMGAGTEGANASYILIRDTHSLRTTAFHGQQVLYWESESSQAEMEFSEGFLKAQASLRGQYWTLQSWVPEMQDPQSWKGKEGT
Domains	DOMAIN 80 286 Peptidase M12B DOMAIN 287 383 Disintegrin DOMAIN 384 439 TSP type-1 1 DOMAIN 682 730 TSP type-1 2 DOMAIN 742 805 TSP type-1 3 DOMAIN 808 859 TSP type-1 4 DOMAIN 896 950 TSP type-1 5 DOMAIN 951 1011 TSP type-1 6 DOMAIN 1012 1068 TSP type-1 7 DOMAIN 1072 1131 TSP type-1 8 DOMAIN 1192 1298 CUB 1 DOMAIN 1299 1427 CUB 2
CUB sequence regions	1192 - 1298 RQHLEPTGTIDMRGPGQADCAVAIGRPLGEVVTLRVLESSLNCSAGDMLLLWGRLTWRKMCRKLLDMTFSSKTNTLVVRQRCGRPGGGVLLRYGSQLAPETFYRECD 1299 - 1427 MQLFGPWGEIVSPSLSPATSNAGGCRLFINVAPHARIAIHALATNMGAGTEGANASYILIRDTHSLRTTAFHGQQVLYWESESSQAEMEFSEGFLKAQASLRGQYWTLQSWVPEMQDPQSWKGKEGT
Function	Cleaves the vWF multimers in plasma into smaller forms thereby controlling vWF-mediated platelet thrombus formation
Active Site	ACT_SITE 225 225
Motifs	MOTIF 498 500 Cell attachment site
Mutation	71 71 R->K: Abolishes pro-domain removal but no loss of proteolytic activity when associated with D-73 73 73 R->D: Abolishes pro-domain removal but no loss of proteolytic activity when associated with K-71 83 83 E->A: No change in calcium dependence for proteolysis 173 173 D->A: No change in calcium dependence for proteolysis 184 184 E->A: Dramatically reduced affinity for calcium 187 187 D->A: Dramatically reduced affinity for calcium 212 212 E->A: Dramatically reduced affinity for calcium 399 399 S->A: No effect on cleavage of VWF and little change in secretion of ADAMTS13 when associated with A-698 698 698 S->A: No effect on cleavage of VWF and greatly reduced secretion of ADAMTS13 when associated with A-399 757 757 S->A: No effect on cleavage of VWF and little change in secretion of ADAMTS13 907 907 S->A: No effect on cleavage of VWF and greatly reduced secretion of ADAMTS13 when associated with A-965 965 965 S->A: No effect on cleavage of VWF and little change in secretion of ADAMTS13 when associated with A-907 1027 1027 S->A: No effect on cleavage of VWF and little change in secretion of ADAMTS13 when associated with A-1087 1087 1087 S->A: No effect on cleavage of VWF and little change in secretion of ADAMTS13 when associated with A-1027
Disease	"DISEASE: Thrombotic thrombocytopenic purpura congenital (TTP) [MIM:274150]: A hematologic disease characterized by hemolytic anemia with fragmentation of erythrocytes, thrombocytopenia, diffuse and non-focal neurologic findings, decreased renal function and fever recessive Note=The disease is caused by mutations affecting the gene represented in this entry"

Q76LX8 Proteins CUB domain Repeats