Recombinant Human ADAMTS13 protein (ab112399)
Key features and details
- Expression system: Wheat germ
- Tags: GST tag N-Terminus
- Suitable for: ELISA, SDS-PAGE, WB
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Product name
Recombinant Human ADAMTS13 protein -
Expression system
Wheat germ -
Accession
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Protein length
Protein fragment -
Animal free
No -
Nature
Recombinant -
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Species
Human -
Sequence
FINVAPHARIAIHALATNMGAGTEGANASYILIRDTHSLRTTAFHGQQVL YWESESSQAEMEFSEGFLKAQASLRGQYWTLQSWVPEMQDPQSWKGKEGT -
Predicted molecular weight
37 kDa including tags -
Amino acids
1328 to 1427 -
Tags
GST tag N-Terminus
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Preparation and Storage
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Alternative names
- A disintegrin and metalloproteinase with thrombospondin motifs 13
- A disintegrin like and metalloprotease (reprolysin type) with thrombospondin type 1 motif 13
- A disintegrin like and metalloprotease with thrombospondin type 1 motif 13
see all -
Function
Cleaves the vWF multimers in plasma into smaller forms. -
Tissue specificity
Plasma. Expressed primarily in liver. -
Involvement in disease
Defects in ADAMTS13 are the cause of thrombotic thrombocytopenic purpura congenital (TTP) [MIM:274150]; also known as Upshaw-Schulman syndrome (USS). A hematologic disease characterized by hemolytic anemia with fragmentation of erythrocytes, thrombocytopenia, diffuse and non-focal neurologic findings, decreased renal function and fever. -
Sequence similarities
Contains 2 CUB domains.
Contains 1 disintegrin domain.
Contains 1 peptidase M12B domain.
Contains 8 TSP type-1 domains. -
Domain
The pro-domain is not required for folding or secretion and does not perform the common function of maintening enzyme latency.
The spacer domain is necessary to recognize and cleave vWF. The C-terminal TSP type-1 and CUB domains may modulate this interaction. -
Post-translational
modificationsMay contain a C-mannosylation site and O-fucosylation sites in the TSP type-1 domains.
The precursor is processed by a furin endopeptidase which cleaves off the pro-domain. -
Cellular localization
Secreted. - Information by UniProt
Images
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SDS-PAGE - Recombinant Human ADAMTS13 protein (ab112399)12.5% SDS-PAGE analysis of ab112399. Stained with Coomassie Blue
