Biochemistry and genetics of von Willebrand factor.
- J. Sadler
- Biology, MedicineAnnual Review of Biochemistry
Growing body of information about VWF synthesis, structure, and function has allowed the reclassification of VWD based upon distinct pathophysiologic mechanisms that appear to correlate with clinical symptoms and the response to therapy.
Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor
Six categories of von Willebrand disease correlate with important clinical features and therapeutic requirements, and certain VWD types, especially type 1 and type 2A, encompass several pathophysiologic mechanisms that sometimes can be distinguished by appropriate laboratory studies.
Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura.
Assays of ADAMTS13 activity and inhibitors in addition to the clinical categories (idiopathic TTP and nonidiopathy TTP) are predictive of outcome and may be useful to tailor patient treatment.
Structure of von Willebrand Factor-cleaving Protease (ADAMTS13), a Metalloprotease Involved in Thrombotic Thrombocytopenic Purpura*
- Xinglong Zheng, D. Chung, T. Takayama, E. Majerus, J. Sadler, K. Fujikawa
- BiologyJournal of Biological Chemistry
- 2 November 2001
The 4.6-kilobase pair cDNA sequence for VWFCP has been determined and may have functional significance, producing proteins with distinct abilities to interact with cofactors, connective tissue, platelets, and von Willebrand factor.
Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura.
- J. Sadler
- Medicine, BiologyBlood
- 1 July 2008
Intensive immunosuppressive therapy with rituximab appears to be effective as salvage therapy, and ongoing clinical trials should determine whether adjuvant ritUXimab with plasma exchange also is beneficial at first diagnosis.
Assembly of Weibel–Palade body-like tubules from N-terminal domains of von Willebrand factor
- Ren-huai Huang, Ying A. Wang, J. Sadler
- BiologyProceedings of the National Academy of Sciences
- 15 January 2008
The symmetry and location of interdomain contacts suggest that decreasing pH along the secretory pathway coordinates the disulfide-linked assembly of VWF multimers with their tubular packaging.
Acquired von Willebrand syndrome: data from an international registry.
It appears that AvWS is especially frequent in lympho- or myeloproliferative and cardiovascular diseases and should be suspected and searched with the appropriate laboratory tests especially when excessive bleeding occurs in patients with these disorders.
A revised classification of von Willebrand disease. For the Subcommittee on von Willebrand Factor of the Scientific and Standardization Committee of the International Society on Thrombosis and…
- J. Sadler
- BiologyThrombosis and Haemostasis
- 1 April 1994
A simplified phenotypic classification of von Willebrand disease is proposed that is based on differences in pathophysiology and standard amino acid and nucleotide numbering schemes are recommended for the description of mutations.
Von Willebrand disease type 1: a diagnosis in search of a disease.
- J. Sadler
- 15 March 2003
Many patients diagnosed with VWD type 1 do not have a specific hemorrhagic disease at all, which limits the utility of the diagnosis, and an empirical epidemiologic approach like that applied to other modest risk factors for disease such as elevated cholesterol and high blood pressure should be substituted.