Annotation Detail

Information
Associated Genes
ADAMTS13
Associated Variants
ADAMTS13 p.His96Asp (p.H96D) ( ENST00000355699.7, ENST00000356589.6, ENST00000371911.7, ENST00000371916.5, ENST00000371929.7 )
ADAMTS13 p.Arg102Cys (p.R102C) ( ENST00000355699.7, ENST00000356589.6, ENST00000371911.7, ENST00000371916.5, ENST00000371929.7 )
ADAMTS13 p.Arg398His (p.R398H) ( ENST00000355699.7, ENST00000356589.6, ENST00000371916.5, ENST00000371929.7 )
ADAMTS13 p.Arg528Gly (p.R528G) ( ENST00000355699.7, ENST00000356589.6, ENST00000371916.5, ENST00000371929.7 )
ADAMTS13 p.Arg692Cys (p.R692C) ( ENST00000355699.7, ENST00000356589.6, ENST00000371916.5, ENST00000371929.7 )
ADAMTS13 p.Cys951Gly (p.C951G) ( ENST00000355699.7, ENST00000356589.6, ENST00000371916.5, ENST00000371929.7 )
ADAMTS13 p.Cys1024Gly (p.C1024G) ( ENST00000355699.7, ENST00000356589.6, ENST00000371916.5, ENST00000371929.7 )
ADAMTS13 p.Cys1213Tyr (p.C1213Y) ( ENST00000355699.7, ENST00000356589.6, ENST00000371910.1, ENST00000371916.5, ENST00000371929.7 )
ADAMTS13 p.His96Asp (p.H96D) ( ENST00000355699.7, ENST00000356589.6, ENST00000371911.7, ENST00000371916.5, ENST00000371929.7 )
ADAMTS13 p.Arg102Cys (p.R102C) ( ENST00000355699.7, ENST00000356589.6, ENST00000371911.7, ENST00000371916.5, ENST00000371929.7 )
ADAMTS13 p.Arg398His (p.R398H) ( ENST00000355699.7, ENST00000356589.6, ENST00000371916.5, ENST00000371929.7 )
ADAMTS13 p.Arg528Gly (p.R528G) ( ENST00000355699.7, ENST00000356589.6, ENST00000371916.5, ENST00000371929.7 )
ADAMTS13 p.Arg692Cys (p.R692C) ( ENST00000355699.7, ENST00000356589.6, ENST00000371916.5, ENST00000371929.7 )
ADAMTS13 p.Cys951Gly (p.C951G) ( ENST00000355699.7, ENST00000356589.6, ENST00000371916.5, ENST00000371929.7 )
ADAMTS13 p.Cys1024Gly (p.C1024G) ( ENST00000355699.7, ENST00000356589.6, ENST00000371916.5, ENST00000371929.7 )
ADAMTS13 p.Cys1213Tyr (p.C1213Y) ( ENST00000355699.7, ENST00000356589.6, ENST00000371910.1, ENST00000371916.5, ENST00000371929.7 )
Associated Disease
Congenital Thrombotic Thrombocytopenic Purpura
Source Database
DisGeNET
Description
We show that deficiency of ADAMTS13 is the molecular mechanism responsible for TTP, and suggest that physiologic proteolysis of VWF and/or other ADAMTS13 substrates is required for normal vascular homeostasis.
Pubmed
11586351
Original source reporting the Gene Disease association
UNIPROT
DisGENET score for the Gene Disease association
0.453224706861814
Year of publication
2001
Drugs