ArticleBlood2007
An IAP retrotransposon in the mouse ADAMTS13 gene creates ADAMTS13 variant proteins that are less effective in cleaving von Willebrand factor multimers.
Article in Blood, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 36 citations in OpenAlex.
- A fully humanized von Willebrand disease type 1 mouse model as unique platform to investigate novel therapeutic options.Haematologica · 2025Article
- Mechanisms of ADAMTS13 regulation.Journal of thrombosis and haemostasis : JTH · 2022Review
- The conserved DNMT1-dependent methylation regions in human cells are vulnerable to neurotoxicant rotenone exposure.Epigenetics & chromatin · 2020Article
- Article
- Mouse germ line mutations due to retrotransposon insertions.Mobile DNA · 2019Review
- A novel role for von Willebrand factor in the pathogenesis of experimental cerebral malaria.Blood · 2016Article
- Nxf1 natural variant E610G is a semi-dominant suppressor of IAP-induced RNA processing defects.PLoS genetics · 2015Article
- Probing ADAMTS13 substrate specificity using phage display.PloS one · 2015Article
- Allosteric activation of ADAMTS13 by von Willebrand factor.Proceedings of the National Academy of Sciences of the United States of America · 2014Article
- Mouse endogenous retroviruses can trigger premature transcriptional termination at a distance.Genome research · 2012Article
- Unraveling the scissile bond: how ADAMTS13 recognizes and cleaves von Willebrand factor.Blood · 2011Review
- Escherichia coli-derived von Willebrand factor-A2 domain fluorescence/Förster resonance energy transfer proteins that quantify ADAMTS13 activity.Analytical biochemistry · 2011Article
- The function of ADAMTS13 in thrombogenesis in vivo: insights from mutant mice.International journal of hematology · 2010Review
- Pathophysiology of thrombotic thrombocytopenic purpura.International journal of hematology · 2010Review
- A novel active endogenous retrovirus family contributes to genome variability in rat inbred strains.Genome research · 2010Article
- Multi-step binding of ADAMTS-13 to von Willebrand factor.Journal of thrombosis and haemostasis : JTH · 2009Article
- An insertion of intracisternal A-particle retrotransposon in a novel member of the phosphoglycerate mutase family in the lew allele of mutant mice.Genes & genetic systems · 2009Article
- A novel binding site for ADAMTS13 constitutively exposed on the surface of globular VWF.Blood · 2009Article
- Positional cloning of zinc finger domain transcription factor Zfp69, a candidate gene for obesity-associated diabetes contributed by mouse locus Nidd/SJL.PLoS genetics · 2009Article
- The distal carboxyl-terminal domains of ADAMTS13 are required for regulation of in vivo thrombus formation.Blood · 2009Article
Corrections and comments
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Authors and funding
3 authors at 2 institutions in 1 country.
Funding
Abstract
Severe deficiency of ADAMTS13, a von Willebrand factor (VWF)-cleaving metalloprotease, causes thrombotic thrombocytopenic purpura. When analyzed with VWF multimers, but not with an abbreviated VWF peptide (VWF73) as the substrate, the plasma ADAMTS13 activity levels of mouse strains segregated into a high and a low group that differed by approximately 10 fold. Low ADAMTS13 activity was detected in mice containing 2 alleles of intracisternal A-type particle (IAP) retrotransposon sequence in the ADAMTS13 gene. Molecular cloning of mouse ADAMTS13 identified 2 truncated variants (IAP-a and IAP-b) in the low-activity mice. Both of the IAP variants lacked the 2 carboxyl terminus thrombospondin type 1 repeat (TSR) and CUB domains of full-length ADAMTS13. The IAP-b variant also had splicing abnormalities affecting the spacer domain sequence and had miniscule enzymatic activity. Compared with full-length ADAMTS13, the IAP-a variant was approximately one ninth as active in cleaving VWF multimers but was only slightly less active in cleaving VWF73 peptide. Recombinant human ADAMTS13 was also less effective in cleaving VWF multimers than VWF73 when the C-terminal TSR sequence was deleted. In summary, the carboxyl terminus TSR sequence is important for cleaving VWF multimers. Assay results should be interpreted with caution when peptide substrates are used for analysis of variant ADAMTS13 proteins.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.