Results 61 to 70 of about 8,735 (203)
Veterinary Clinical Pathology, EarlyView.ABSTRACT Background In humans, deficiency of a disintegrin‐like and metalloprotease with thrombospondin type 1 motifs, number 13 (ADAMTS13), a von Willebrand factor (vWF) cleaving protease, is an underlying mechanism for thrombosis. The role of ADAMTS13 in canine thromboembolic disease is largely unknown.
Ashley R. Wilkinson +2 more
wiley +1 more source
Detection of Plasma Protease Activity Using Microsphere-Cytometry Assays with E. coli Derived Substrates: VWF Proteolysis by ADAMTS13. [PDF]
PLoS ONE, 2015 Protease levels in human blood are often prognostic indicators of inflammatory, thrombotic or oncogenic disorders. The measurement of such enzyme activities in substrate-based assays is complicated due to the low prevalence of these enzymes and steric ...
Shobhit Gogia +2 more
doaj +1 more source
Multi-lectin Affinity Chromatography and Quantitative Proteomic Analysis Reveal Differential Glycoform Levels between Prostate Cancer and Benign Prostatic Hyperplasia Sera. [PDF]
, 2018 Currently prostate-specific antigen is used for prostate cancer (PCa) screening, however it lacks the necessary specificity for differentiating PCa from other diseases of the prostate such as benign prostatic hyperplasia (BPH), presenting a clinical need
Adusumilli, Ravali +6 more
core +2 more sources
Journal of Inherited Metabolic Disease, Volume 49, Issue 2, March 2026.ABSTRACT The vital cofactors NADH and NADPH are prone to hydration, forming hydroxylated redox‐inactive derivatives (NADHX and NADPHX) in cells. These damaged metabolites are repaired by two highly conserved enzymes, an NAD(P)HX dehydratase (NAXD) and an NAD(P)HX epimerase (NAXE).
Myrto Patraskaki +6 more
wiley +1 more source
PROTEOLYTIC PROCESSING OF VON WILLEBRAND FACTOR BY ADAMTS13 AND LEUKOCYTE PROTEASES
Mediterranean Journal of Hematology and Infectious Diseases, 2013 ADAMTS13 is a 190 kDa zinc protease encoded by a gene located on chromosome 9q34. This protease specifically hydrolyzes von Willebrand factor (VWF) multimers, thus causing VWF size reduction.
Stefano Lancellotti +2 more
doaj +1 more source
IKAROS Associated Immunodeficiency and Thrombotic Thrombocytopenic Purpura
Pediatric Blood &Cancer, Volume 73, Issue 2, February 2026.ABSTRACT Pathogenic variants in IKZF1 (IKAROS) are linked to immunodeficiency, malignancy, and immune dysregulation. We describe a family with a rare IKZF1 variant presenting with humoral immunodeficiency and thrombotic thrombocytopenic purpura (TTP). A non‐consanguineous family was clinically monitored; clinical, immunological, and genetic data (exome
Ilia Spivak +7 more
wiley +1 more source
Critical Care, 2020 Background A dysbalanced coagulation system is part of the pathological host response to infection in sepsis. Activation of pro-coagulant pathways and attenuation of anti-coagulant activity ultimately lead to microvascular stasis and consequent organ ...
Klaus Stahl +9 more
doaj +1 more source
Structural basis of type 2A von Willebrand disease investigated by molecular dynamics simulations and experiments. [PDF]
PLoS ONE, 2012 The hemostatic function of von Willebrand factor is downregulated by the metalloprotease ADAMTS13, which cleaves at a unique site normally buried in the A2 domain.
Gianluca Interlandi +4 more
doaj +1 more source
Conformational activation of ADAMTS13 [PDF]
, 2014 A disintegrin and metalloprotease with thrombospondin motifs 13 (ADAMTS13) is a metalloprotease that regulates von Willebrand factor (VWF) function. ADAMTS13-mediated proteolysis is determined by conformational changes in VWF, but also may depend on its ...
Collins, RF +8 more
core +1 more source
Molecular Genetics &Genomic Medicine, Volume 14, Issue 1, January 2026.This study reports a rare case of bone marrow failure syndrome type 1 (BMFS1) caused by a novel de novo splicing mutation (c.1502+1G>A) in the SRP72 gene. The 6‐year‐old patient presented with aplastic anemia and pancytopenia. Genetic analysis identified the mutation, which was absent in both parents, confirming its de novo origin.
Wang Xiangwen +3 more
wiley +1 more source