General Information of the Ferroptosis Regulator (ID: REG10250)
Regulator Name A disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAMTS13)
Synonyms
C9orf8; von Willebrand factor-cleaving protease
    Click to Show/Hide
Gene Name ADAMTS13
Gene ID 11093
Regulator Type Protein coding
Uniprot ID Q76LX8
Sequence
MHQRHPRARCPPLCVAGILACGFLLGCWGPSHFQQSCLQALEPQAVSSYLSPGAPLKGRP
PSPGFQRQRQRQRRAAGGILHLELLVAVGPDVFQAHQEDTERYVLTNLNIGAELLRDPSL
GAQFRVHLVKMVILTEPEGAPNITANLTSSLLSVCGWSQTINPEDDTDPGHADLVLYITR
FDLELPDGNRQVRGVTQLGGACSPTWSCLITEDTGFDLGVTIAHEIGHSFGLEHDGAPGS
GCGPSGHVMASDGAAPRAGLAWSPCSRRQLLSLLSAGRARCVWDPPRPQPGSAGHPPDAQ
PGLYYSANEQCRVAFGPKAVACTFAREHLDMCQALSCHTDPLDQSSCSRLLVPLLDGTEC
GVEKWCSKGRCRSLVELTPIAAVHGRWSSWGPRSPCSRSCGGGVVTRRRQCNNPRPAFGG
RACVGADLQAEMCNTQACEKTQLEFMSQQCARTDGQPLRSSPGGASFYHWGAAVPHSQGD
ALCRHMCRAIGESFIMKRGDSFLDGTRCMPSGPREDGTLSLCVSGSCRTFGCDGRMDSQQ
VWDRCQVCGGDNSTCSPRKGSFTAGRAREYVTFLTVTPNLTSVYIANHRPLFTHLAVRIG
GRYVVAGKMSISPNTTYPSLLEDGRVEYRVALTEDRLPRLEEIRIWGPLQEDADIQVYRR
YGEEYGNLTRPDITFTYFQPKPRQAWVWAAVRGPCSVSCGAGLRWVNYSCLDQARKELVE
TVQCQGSQQPPAWPEACVLEPCPPYWAVGDFGPCSASCGGGLRERPVRCVEAQGSLLKTL
PPARCRAGAQQPAVALETCNPQPCPARWEVSEPSSCTSAGGAGLALENETCVPGADGLEA
PVTEGPGSVDEKLPAPEPCVGMSCPPGWGHLDATSAGEKAPSPWGSIRTGAQAAHVWTPA
AGSCSVSCGRGLMELRFLCMDSALRVPVQEELCGLASKPGSRREVCQAVPCPARWQYKLA
ACSVSCGRGVVRRILYCARAHGEDDGEEILLDTQCQGLPRPEPQEACSLEPCPPRWKVMS
LGPCSASCGLGTARRSVACVQLDQGQDVEVDEAACAALVRPEASVPCLIADCTYRWHVGT
WMECSVSCGDGIQRRRDTCLGPQAQAPVPADFCQHLPKPVTVRGCWAGPCVGQGTPSLVP
HEEAAAPGRTTATPAGASLEWSQARGLLFSPAPQPRRLLPGPQENSVQSSACGRQHLEPT
GTIDMRGPGQADCAVAIGRPLGEVVTLRVLESSLNCSAGDMLLLWGRLTWRKMCRKLLDM
TFSSKTNTLVVRQRCGRPGGGVLLRYGSQLAPETFYRECDMQLFGPWGEIVSPSLSPATS
NAGGCRLFINVAPHARIAIHALATNMGAGTEGANASYILIRDTHSLRTTAFHGQQVLYWE
SESSQAEMEFSEGFLKAQASLRGQYWTLQSWVPEMQDPQSWKGKEGT

    Click to Show/Hide
Function
Cleaves the vWF multimers in plasma into smaller forms thereby controlling vWF-mediated platelet thrombus formation.

    Click to Show/Hide
HGNC ID
HGNC:1366
KEGG ID hsa:11093
Full List of the Ferroptosis Target of This Regulator and Corresponding Disease/Drug Response(s)
ADAMTS13 can regulate the following target(s), and cause disease/drug response(s). You can browse detail information of target(s) or disease/drug response(s).
Browse Target
Browse Disease
Nuclear factor erythroid 2-related factor 2 (NFE2L2) [Suppressor; Marker]
In total 1 item(s) under this target
Experiment 1 Reporting the Ferroptosis Target of This Regulator [1]
Target for Ferroptosis Marker/Suppressor
Responsed Disease Acute kidney failure ICD-11: GB60
Pathway Response Fatty acid metabolism hsa01212
Ferroptosis hsa04216
Cell Process Cell ferroptosis
In Vitro Model
mKTs (Mouse knee tissues)
In Vivo Model
C57BL/6 mice (8-12 week) mice purchased from Cavens (Changzhou, China) were divided into four groups: control (saline), CDDP only (CP, 20 mg/kg; MCE, United States), CP (20 mg/kg) + ADAMTS13 (0.1 nmol/kg) and CP (20 mg/kg) + ADAMTS13 (0.3 nmol/kg). Each group contained 5 mice. 0.1 and 0.3 nmol/kg rhADAMTS13 were injected into the caudal vein daily for 3 days after surgery.

    Click to Show/Hide
Response regulation ADAMTS13 alleviated CP-induced inflammatory response and oxidative stress in acute kidney injury mice, during which the Nrf2 signaling pathway was abnormal. Overall, ADAMTS-13-regulated Nrf2 signaling inhibits ferroptosis to ameliorate CP-induced AKI.
Acute kidney failure [ICD-11: GB60]
In total 1 item(s) under this disease
Experiment 1 Reporting the Ferroptosis-centered Disease Response [1]
Target Regulator A disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAMTS13) Protein coding
Pathway Response Fatty acid metabolism hsa01212
Ferroptosis hsa04216
Cell Process Cell ferroptosis
In Vitro Model
mKTs (Mouse knee tissues)
In Vivo Model
C57BL/6 mice (8-12 week) mice purchased from Cavens (Changzhou, China) were divided into four groups: control (saline), CDDP only (CP, 20 mg/kg; MCE, United States), CP (20 mg/kg) + ADAMTS13 (0.1 nmol/kg) and CP (20 mg/kg) + ADAMTS13 (0.3 nmol/kg). Each group contained 5 mice. 0.1 and 0.3 nmol/kg rhADAMTS13 were injected into the caudal vein daily for 3 days after surgery.

    Click to Show/Hide
Response regulation ADAMTS13 alleviated CP-induced inflammatory response and oxidative stress in acute kidney injury mice, during which the Nrf2 signaling pathway was abnormal. Overall, ADAMTS-13-regulated Nrf2 signaling inhibits ferroptosis to ameliorate CP-induced AKI.
References
Ref 1 ADAMTS-13-regulated nuclear factor E2-related factor 2 signaling inhibits ferroptosis to ameliorate cisplatin-induced acute kidney injuy. Bioengineered. 2021 Dec;12(2):11610-11621. doi: 10.1080/21655979.2021.1994707.