Property Summary

NCBI Gene PubMed Count 366
Grant Count 242
R01 Count 136
Funding $30,646,811.43
PubMed Score 1300.80
PubTator Score 1273.38

Knowledge Summary

Patent

No data available

Expression

  Differential Expression (1)

Disease log2 FC p
osteosarcoma 1.120 0.000

Synonym

Accession Q76LX8 Q6UY16 Q710F6 Q711T8 Q96L37 Q9H0G3 Q9UGQ1 ADAM-TS 13
Symbols VWFCP
C9orf8
vWF-CP
ADAM-TS13
ADAMTS-13

Gene

PDB

3GHM   3GHN   3VN4  

Gene RIF (341)

PMID Text
26897861 ADAMTS13 is a von Willebrand factor-cleaving protease and its deficiency causes thrombotic thrombocytopenic purpura.
26773038 Glycan stabilization of the VWF A2 domain acts together with the Ca(2+)binding site and vicinal cysteine disulfide bond to control unfolding and ADAMTS13 proteolysis.
26747250 These data provide evidence for the involvement of CUB2 domain of ADAMTS13 - reactive CD4(+)T cells in the etiology of acquired thrombotic thrombocytopenic purpura.
26616301 An in vitro model for LVAD associated aVWD demonstrated that ADAMTS-13 and platelets contribute to the depletion of HMWM of VWF.
26595154 ADAMTS13 is the key protease that regulates the multimeric state of VWF. Without ADAMTS13, VWF multimers can grow to pathologically large sizes. This is a risk factor for the life-threatening condition thrombotic thrombocytopenic purpura (TTP)
26456149 Implementation of a rapid turnaround assay for ADAMTS13 resulted in a significant reduction in plasma utilization for patients with suspected TTP, without an increase in mortality
26454844 ADAMTS-13 is a clinical target to reduce vWF degradation, improve vWF function, and potentially reduce bleeding during LVAD support.
26425683 Disappearing acts of ADAMTS13.
26391536 ADAMTS-13 conformational activation leads to cryptic epitope/exosite exposure in proximal and distal domains, increasing activity. 3 linker regions in the distal domains make it flexible, enabling the interaction of the proximal and the T8-CUB2 domains.
26345337 Type 2B mutations localized in the A1 domain could enhance the sensitivity to ADAMTS13-mediated proteolysis. When GPIbalpha participated, there was a dramatically increased proteolytic cleavage of VWF by ADAMTS13 to rVWF-WT, excluding some type 2B mutants.
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AA Sequence

MHQRHPRARCPPLCVAGILACGFLLGCWGPSHFQQSCLQALEPQAVSSYLSPGAPLKGRPPSPGFQRQRQ      1 - 70
RQRRAAGGILHLELLVAVGPDVFQAHQEDTERYVLTNLNIGAELLRDPSLGAQFRVHLVKMVILTEPEGA     71 - 140
PNITANLTSSLLSVCGWSQTINPEDDTDPGHADLVLYITRFDLELPDGNRQVRGVTQLGGACSPTWSCLI    141 - 210
TEDTGFDLGVTIAHEIGHSFGLEHDGAPGSGCGPSGHVMASDGAAPRAGLAWSPCSRRQLLSLLSAGRAR    211 - 280
CVWDPPRPQPGSAGHPPDAQPGLYYSANEQCRVAFGPKAVACTFAREHLDMCQALSCHTDPLDQSSCSRL    281 - 350
LVPLLDGTECGVEKWCSKGRCRSLVELTPIAAVHGRWSSWGPRSPCSRSCGGGVVTRRRQCNNPRPAFGG    351 - 420
RACVGADLQAEMCNTQACEKTQLEFMSQQCARTDGQPLRSSPGGASFYHWGAAVPHSQGDALCRHMCRAI    421 - 490
GESFIMKRGDSFLDGTRCMPSGPREDGTLSLCVSGSCRTFGCDGRMDSQQVWDRCQVCGGDNSTCSPRKG    491 - 560
SFTAGRAREYVTFLTVTPNLTSVYIANHRPLFTHLAVRIGGRYVVAGKMSISPNTTYPSLLEDGRVEYRV    561 - 630
ALTEDRLPRLEEIRIWGPLQEDADIQVYRRYGEEYGNLTRPDITFTYFQPKPRQAWVWAAVRGPCSVSCG    631 - 700
AGLRWVNYSCLDQARKELVETVQCQGSQQPPAWPEACVLEPCPPYWAVGDFGPCSASCGGGLRERPVRCV    701 - 770
EAQGSLLKTLPPARCRAGAQQPAVALETCNPQPCPARWEVSEPSSCTSAGGAGLALENETCVPGADGLEA    771 - 840
PVTEGPGSVDEKLPAPEPCVGMSCPPGWGHLDATSAGEKAPSPWGSIRTGAQAAHVWTPAAGSCSVSCGR    841 - 910
GLMELRFLCMDSALRVPVQEELCGLASKPGSRREVCQAVPCPARWQYKLAACSVSCGRGVVRRILYCARA    911 - 980
HGEDDGEEILLDTQCQGLPRPEPQEACSLEPCPPRWKVMSLGPCSASCGLGTARRSVACVQLDQGQDVEV    981 - 1050
DEAACAALVRPEASVPCLIADCTYRWHVGTWMECSVSCGDGIQRRRDTCLGPQAQAPVPADFCQHLPKPV   1051 - 1120
TVRGCWAGPCVGQGTPSLVPHEEAAAPGRTTATPAGASLEWSQARGLLFSPAPQPRRLLPGPQENSVQSS   1121 - 1190
ACGRQHLEPTGTIDMRGPGQADCAVAIGRPLGEVVTLRVLESSLNCSAGDMLLLWGRLTWRKMCRKLLDM   1191 - 1260
TFSSKTNTLVVRQRCGRPGGGVLLRYGSQLAPETFYRECDMQLFGPWGEIVSPSLSPATSNAGGCRLFIN   1261 - 1330
VAPHARIAIHALATNMGAGTEGANASYILIRDTHSLRTTAFHGQQVLYWESESSQAEMEFSEGFLKAQAS   1331 - 1400
LRGQYWTLQSWVPEMQDPQSWKGKEGT                                              1401 - 1427
//

Text Mined References (367)

PMID Year Title
26897861 2015 [Thrombotic Thrombocytopenic Purpura --Pathophysiology and Assays of ADAMTS13 Activity].
26773038 2016 N-linked glycan stabilization of the VWF A2 domain.
26747250 2016 CD4+ T cells from patients with acquired thrombotic thrombocytopenic purpura recognize CUB2 domain-derived peptides.
26616301 2016 Acquired von Willebrand factor deficiency caused by LVAD is ADAMTS-13 and platelet dependent.
26595154 2016 Keeping von Willebrand Factor under Control: Alternatives for ADAMTS13.
26456149 2016 Effect of ADAMTS13 activity turnaround time on plasma utilization for suspected thrombotic thrombocytopenic purpura.
26454844 2015 Inhibition of ADAMTS-13 by Doxycycline Reduces von Willebrand Factor Degradation During Supraphysiological Shear Stress: Therapeutic Implications for Left Ventricular Assist Device-Associated Bleeding.
26425683 2015 Disappearing acts of ADAMTS13.
26391536 2015 Linker regions and flexibility around the metalloprotease domain account for conformational activation of ADAMTS-13.
26345337 2015 The co-influence of VWD type 2B/2M mutations in the A1 domain and platelet GPIb? on the rate of cleavage to VWF by ADAMTS13.
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