Human neutrophil peptides inhibit cleavage of von Willebrand factor by ADAMTS13: a potential link of inflammation to TTP-Reference-Cited by-同舟云学术

Human neutrophil peptides inhibit cleavage of von Willebrand factor by ADAMTS13: a potential link of inflammation to TTP

Published:2016-07-07 Issue:1 Volume:128 Page:110-119
ISSN:0006-4971
Container-title:Blood
language:en
Short-container-title:

Author:

Pillai Vikram G.¹,Bao Jialing²,Zander Catherine B.¹,McDaniel Jenny K.³,Chetty Palaniappan S.²,Seeholzer Steven H.²,Bdeir Khalil⁴,Cines Douglas B.⁴^ORCID,Zheng X. Long¹^ORCID

Affiliation:

1. Division of Laboratory Medicine, Department of Pathology, University of Alabama at Birmingham, Birmingham, AL;

2. Department of Pathology and Laboratory Medicine, Children’s Hospital of Philadelphia, Philadelphia, PA;

3. Division of Pediatric Hematology/Oncology, Children’s Hospital of Alabama, Birmingham, AL; and

4. Department of Pathology and Laboratory Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA

Abstract

Key Points HNPs inhibit proteolytic cleavage of VWF by ADAMTS13 by physically blocking VWF-ADAMTS13 interactions. Plasma levels of HNP1, HNP2, and HNP3 are markedly increased in patients with acquired autoimmune TTP.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Link

http://ashpublications.org/blood/article-pdf/128/1/110/1395598/110.pdf

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5. Prognostic value of anti-ADAMTS 13 antibody features (Ig isotype, titer, and inhibitory effect) in a cohort of 35 adult French patients undergoing a first episode of thrombotic microangiopathy with undetectable ADAMTS 13 activity.;Ferrari;Blood,2007

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