Results 41 to 50 of about 3,354,179 (197)

Von Willebrand Factor and ADAMTS13 in COVID-19 and Beyond: A Question of Balance

European Medical Journal Nephrology, 2021
von Willebrand factor (VWF) is a large, adhesive, multimeric protein involved in haemostasis. The larger the size (or number of VWF multimers), the greater the functionality of the protein. A deficiency or defect in VWF can lead to von Willebrand disease
Emmanuel J Favaloro, Brandon Michael Henry, Giuseppe Lippi   +2 more
doaj  

Three disintegrin-like domain mutations of ADAMTS13： functional deficiency and association with thrombosis [PDF]

Zhenduanxue lilun yu shijian
Objective To analyze the genotypic and phenotypic characteristics of four thrombotic patients carrying heterozygous mutations in the disintegrin-like domain of ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motifs 13), and to ...
LIN Liya, WU Xi, MAO Yinqi, CHEN Guangming, WU Wenman, DAI Jing, WANG Xuefeng, DING Qiulan
doaj   +1 more source

Escherichia coli-derived von Willebrand factor-A2 domain fluorescence/Förster resonance energy transfer proteins that quantify ADAMTS13 activity [PDF]

Analytical Biochemistry, 2011
The cleavage of the A2 domain of von Willebrand factor (VWF) by the metalloprotease ADAMTS13 regulates VWF size and platelet thrombosis rates. Reduction or inhibition of this enzyme activity leads to thrombotic thrombocytopenic purpura (TTP). We generated a set of novel molecules called VWF-A2 FRET (fluorescence/Förster resonance energy transfer ...
Kannayakanahalli M, Dayananda, Shobhit, Gogia, Sriram, Neelamegham   +2 more
openaire   +2 more sources

ADAMTS13 activity and antigen during therapy and follow-up of patients with idiopathic thrombotic thrombocytopenic purpura: correlation with clinical outcome

Haematologica, 2011
Background The assay for ADAMTS13 activity helps clinicians to confirm the clinical diagnosis of idiopathic thrombotic thrombocytopenic purpura. The clinical value of testing for the antigen level of ADAMTS13 protein is, however, less clear.Design and ...
Shangbin Yang, Ming Jin, Shili Lin, Spero Cataland, Haifeng Wu   +4 more
doaj   +1 more source

CHAPTER 1.1. Disulfide Bonds in Protein Folding and Stability [PDF]

, 2018
Disulfide bonds are unique among post-translational modifications, as they add covalent crosslinks to the polypeptide chain. Accordingly, they can exert pronounced effects on protein folding and stability. This is of particular importance for secreted or
Sub Cellular Protein Chemistry, Hendershot, Linda M., Feige, Matthias J., Ineke Braakman, Matthias J. Feige, Braakman, Ineke, Cellular Protein Chemistry, Linda M. Hendershot   +7 more
core   +1 more source

The role of von Willebrand factor and its cleaving protease, ADAMTS13, in young patients with HIV-related stroke. [PDF]

, 2013
Includes abstract.Includes bibliographical references.The Human Immunodeficiency Virus (HIV) is neuro-invasive and neurological complications of HIV infection occur frequently through a variety of possible mechanisms.
Allie, Sameera
core   +1 more source

Antibodies against the CUB1-2 domains of ADAMTS13 in a patient with benign monoclonal gammopathy: no causal relationship

Haematologica, 2007
We present a patient with a history of benign monoclonal gammopathy, who developed thrombotic thrombocytopenic purpura (TTP), initially presenting as bilateral serous retinal detachment.
Niels P Riksen, Brenda M Luken, Ina S Klasen, Jan Voorberg, Niels Crama, Marcel van Deuren   +5 more
doaj   +1 more source

The first deletion mutation in the TSP1-6 repeat domain of ADAMTS13 in a family with inherited thrombotic thrombocytopenic purpura

Haematologica, 2009
The inherited deficiency of ADAMTS13 is usually associated with severe forms of thrombotic thrombocytopenic purpura. Among the mutations identified in the ADAMTS13 gene, none have been described on the TSP1-6 repeat domain.
Roberta Palla, Silvia Lavoretano, Rossana Lombardi, Isabella Garagiola, Mehran Karimi, Abdolreza Afrasiabi, Mani Ramzi, Raimondo De Cristofaro, Flora Peyvandi   +8 more
doaj   +1 more source

Hereditary Thrombotic Thrombocytopenic Purpura in a Chinese Boy With a Novel Compound Heterozygous Mutation of the ADAMTS13 Gene

Frontiers in Pediatrics, 2020
Hereditary thrombotic thrombocytopenic purpura (TTP) is caused by ADAMTS13 mutations with autosomal recessive inheritance. It typically presents during childhood and is frequently misdiagnosed as immune thrombocytopenia.
Yi-ling Dai, Yi-ling Dai, Xue Tang, Xue Tang, Hong-bo Chen, Hong-bo Chen, Qiu-yu Peng, Qiu-yu Peng, Xia Guo, Xia Guo, Ju Gao, Ju Gao   +11 more
doaj   +1 more source

Unbiased plasma proteomics reveals hemolysis during ADAMTS13 relapse in patients with immune TTP

Blood Advances
: Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy that is caused by a deficiency of a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13 (ADAMTS13).
Tim Postmus, Maryam O. Subhan, Eva R. Smit, Jan Voorberg, Mari Thomas, Maartje van den Biggelaar, Marie Scully, Rens de Groot, Pieter Langerhorst   +8 more
doaj   +1 more source