Evidence map›Paper›PMID 17426255›Full record

ArticleBlood2007

An IAP retrotransposon in the mouse ADAMTS13 gene creates ADAMTS13 variant proteins that are less effective in cleaving von Willebrand factor multimers.

Wenhua Zhou, Eric E Bouhassira, Han-Mou Tsai

Open access · bronzeAbstract readComparative Study
In one paragraph

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.

0numbers the graph read from it
0cells of the map it votes in
25citing papers in PubMed
2.2field-weighted citation impact, top 12% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

25 citing papers in PubMed, 36 citations in OpenAlex.

  1. Article
  2. Mechanisms of ADAMTS13 regulation.Journal of thrombosis and haemostasis : JTH · 2022
    Review
  3. Article
  4. Article
  5. Review
  6. Article
  7. Article
  8. Article
  9. Allosteric activation of ADAMTS13 by von Willebrand factor.Proceedings of the National Academy of Sciences of the United States of America · 2014
    Article
  10. Article
  11. Review
  12. Article
  13. Review
  14. Pathophysiology of thrombotic thrombocytopenic purpura.International journal of hematology · 2010
    Review
  15. Article
  16. Multi-step binding of ADAMTS-13 to von Willebrand factor.Journal of thrombosis and haemostasis : JTH · 2009
    Article
  17. Article
  18. Article
  19. Article
  20. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

3 authors at 2 institutions in 1 country.

Wenhua ZhouDivision of Hematology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, NY 10467, USA.
Eric E Bouhassira
Han-Mou Tsai
Montefiore Medical Center · USAlbert Einstein College of Medicine · US

Funding

VWF PROTEINASE DEFICIENCY IN TTP WITH OR WITHOUT HIVR01HL062136 · NHLBI · PENNSYLVANIA STATE UNIV HERSHEY MED CTR · PI TSAI, HAN-MOU · 1999 to 2008
$2.4M
NHLBI NIH HHS R01 HL062136NHLBI NIH HHS R01 HL062136-07NHLBI NIH HHS R01 HL 62136
6 · The paper itself

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.

Indexed as

Genes, Intracisternal A-ParticleMutagenesis, InsertionalRNA SplicingADAM ProteinsADAMTS13 ProteinAllelesAnimalsCloning, MolecularHumansMetalloendopeptidasesMiceMultiprotein ComplexesPeptidesProtein Structure, TertiaryPurpura, Thrombotic ThrombocytopenicRecombinant ProteinsADAM ProteinsADAMTS13 ProteinADAMTS13 protein, humanADAMTS13 protein, mouseMetalloendopeptidasesMultiprotein ComplexesPeptidesRecombinant Proteinsvon Willebrand Factor

Identifiers

PMID17426255
PMCPMC1924774
OpenAlexW2069779742

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

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.