S L Haberichter

Summary

Affiliation: Medical College of Wisconsin
Country: USA

Publications

  1. pmc The mutation N528S in the von Willebrand factor (VWF) propeptide causes defective multimerization and storage of VWF
    Sandra L Haberichter
    Department of Pediatrics Hematology Oncology, Medical College of Wisconsin and Children s Research Institute, Children s Hospital of Wisconsin, Milwaukee, WI 53226, USA
    Blood 115:4580-7. 2010
  2. pmc Genetic alteration of the D2 domain abolishes von Willebrand factor multimerization and trafficking into storage
    S L Haberichter
    Department of Pediatrics, Medical College of Wisconsin, and Children s Research Institute, Children s Hospital of Wisconsin, Milwaukee, WI 53226, USA
    J Thromb Haemost 7:641-50. 2009
  3. pmc Identification of type 1 von Willebrand disease patients with reduced von Willebrand factor survival by assay of the VWF propeptide in the European study: molecular and clinical markers for the diagnosis and management of type 1 VWD (MCMDM-1VWD)
    Sandra L Haberichter
    Department of Pediatrics, Medical College of Wisconsin, Milwaukee, USA
    Blood 111:4979-85. 2008
  4. pmc Assay of the von Willebrand factor (VWF) propeptide to identify patients with type 1 von Willebrand disease with decreased VWF survival
    Sandra L Haberichter
    Department of Pediatrics Hematology Oncology, Medical College of Wisconsin, 8701 Watertown Plank Rd, Milwaukee, WI 53226, USA
    Blood 108:3344-51. 2006
  5. pmc Variability in platelet- and collagen-binding defects in type 2M von Willebrand disease
    D M Larsen
    Medical College of Wisconsin, Milwaukee, WI, USA
    Haemophilia 19:590-4. 2013
  6. pmc Contribution of platelet vs. endothelial VWF to platelet adhesion and hemostasis
    S Kanaji
    Blood Research Institute, BloodCenter of Wisconsin, Department of Pediatrics, Medical College of Wisconsin, Children s Research Institute, Children s Hospital of Wisconsin, Milwaukee, WI 53226, USA
    J Thromb Haemost 10:1646-52. 2012
  7. pmc Comparison of type I, type III and type VI collagen binding assays in diagnosis of von Willebrand disease
    V H Flood
    Department of Pediatrics, Division of Hematology Oncology, Medical College of Wisconsin, Milwaukee, WI, USA
    J Thromb Haemost 10:1425-32. 2012
  8. pmc Critical von Willebrand factor A1 domain residues influence type VI collagen binding
    V H Flood
    Department of Pediatrics, Division of Hematology Oncology, Medical College of Wisconsin, Milwaukee, WI 53201 2178, USA
    J Thromb Haemost 10:1417-24. 2012
  9. ncbi request reprint Regulated release of VWF and FVIII and the biologic implications
    S L Haberichter
    Department of Pediatrics, Medical College of Wisconsin and Blood Research Institute, Blood Center of Wisconsin, Milwaukee, Wisconsin 53201, USA
    Pediatr Blood Cancer 46:547-53. 2006
  10. ncbi request reprint Induction of megakaryocytes to synthesize and store a releasable pool of human factor VIII
    D A Wilcox
    Department of Pediatrics, Medical College of Wisconsin, and Children s Hospital of Wisconsin, Milwaukee, Wisconsin 53226, USA
    J Thromb Haemost 1:2477-89. 2003

Collaborators

Detail Information

Publications21

  1. pmc The mutation N528S in the von Willebrand factor (VWF) propeptide causes defective multimerization and storage of VWF
    Sandra L Haberichter
    Department of Pediatrics Hematology Oncology, Medical College of Wisconsin and Children s Research Institute, Children s Hospital of Wisconsin, Milwaukee, WI 53226, USA
    Blood 115:4580-7. 2010
    ..In addition, we have identified a potentially novel pathogenic mechanism of VWD, namely a transportation and storage defect of mature VWF due to defective interaction with its transporter, the mutant propeptide...
  2. pmc Genetic alteration of the D2 domain abolishes von Willebrand factor multimerization and trafficking into storage
    S L Haberichter
    Department of Pediatrics, Medical College of Wisconsin, and Children s Research Institute, Children s Hospital of Wisconsin, Milwaukee, WI 53226, USA
    J Thromb Haemost 7:641-50. 2009
    ..Although our laboratory and others have identified mutations in von Willebrand disease patients that disrupt VWF multimerization, little is known about the affect of mutations on the regulated storage of VWF...
  3. pmc Identification of type 1 von Willebrand disease patients with reduced von Willebrand factor survival by assay of the VWF propeptide in the European study: molecular and clinical markers for the diagnosis and management of type 1 VWD (MCMDM-1VWD)
    Sandra L Haberichter
    Department of Pediatrics, Medical College of Wisconsin, Milwaukee, USA
    Blood 111:4979-85. 2008
    ..The systematic assay of both plasma VWF and the VWF propeptide in moderately severe type 1 VWD patients may identify patients with a reduced VWF survival phenotype...
  4. pmc Assay of the von Willebrand factor (VWF) propeptide to identify patients with type 1 von Willebrand disease with decreased VWF survival
    Sandra L Haberichter
    Department of Pediatrics Hematology Oncology, Medical College of Wisconsin, 8701 Watertown Plank Rd, Milwaukee, WI 53226, USA
    Blood 108:3344-51. 2006
    ..This phenotype may require an altered clinical therapeutic approach, and we propose to refer to this phenotype as type-1C VWD...
  5. pmc Variability in platelet- and collagen-binding defects in type 2M von Willebrand disease
    D M Larsen
    Medical College of Wisconsin, Milwaukee, WI, USA
    Haemophilia 19:590-4. 2013
    ..Type 2M VWD is a heterogeneous category comprised of both collagen- and platelet-binding defects. Understanding the precise defect for each mutation may ultimately lead to better diagnosis and treatment...
  6. pmc Contribution of platelet vs. endothelial VWF to platelet adhesion and hemostasis
    S Kanaji
    Blood Research Institute, BloodCenter of Wisconsin, Department of Pediatrics, Medical College of Wisconsin, Children s Research Institute, Children s Hospital of Wisconsin, Milwaukee, WI 53226, USA
    J Thromb Haemost 10:1646-52. 2012
    ..The relative contribution of VWF stored in ECs or megakaryocytes/platelets or present in plasma to hemostasis is not clear...
  7. pmc Comparison of type I, type III and type VI collagen binding assays in diagnosis of von Willebrand disease
    V H Flood
    Department of Pediatrics, Division of Hematology Oncology, Medical College of Wisconsin, Milwaukee, WI, USA
    J Thromb Haemost 10:1425-32. 2012
    ..Collagen binding assays (VWF:CB), however, are not part of the routine work-up for von Willebrand disease (VWD)...
  8. pmc Critical von Willebrand factor A1 domain residues influence type VI collagen binding
    V H Flood
    Department of Pediatrics, Division of Hematology Oncology, Medical College of Wisconsin, Milwaukee, WI 53201 2178, USA
    J Thromb Haemost 10:1417-24. 2012
    ..Laboratory testing for von Willebrand disease (VWD), however, does not always include collagen binding assays (VWF:CB) and standard VWF:CB assays use type I and/or type III collagen rather than type VI collagen...
  9. ncbi request reprint Regulated release of VWF and FVIII and the biologic implications
    S L Haberichter
    Department of Pediatrics, Medical College of Wisconsin and Blood Research Institute, Blood Center of Wisconsin, Milwaukee, Wisconsin 53201, USA
    Pediatr Blood Cancer 46:547-53. 2006
    ..The data presented indicate that regulated secretion of both proteins occurs only if there is endogenous synthesis of FVIII together with VWF...
  10. ncbi request reprint Induction of megakaryocytes to synthesize and store a releasable pool of human factor VIII
    D A Wilcox
    Department of Pediatrics, Medical College of Wisconsin, and Children s Hospital of Wisconsin, Milwaukee, Wisconsin 53226, USA
    J Thromb Haemost 1:2477-89. 2003
    ..These results suggest feasibility for the development of a locally inducible secretory pool of FVIII in platelets of patients with hemophilia A...
  11. pmc Common VWF exon 28 polymorphisms in African Americans affecting the VWF activity assay by ristocetin cofactor
    Veronica H Flood
    Department of Pediatrics, Division of Hematology Oncology, Medical College of Wisconsin, Milwaukee, WI 53201, USA
    Blood 116:280-6. 2010
    ..Because the VWF:RCo assay depends on ristocetin binding to VWF, mutations (and polymorphisms) in VWF may affect the measurement of "VWF activity" by this assay and may not reflect a functional defect or true hemorrhagic risk...
  12. ncbi request reprint Critical independent regions in the VWF propeptide and mature VWF that enable normal VWF storage
    Sandra L Haberichter
    Department of Pediatrics, Medical College of Wisconsin, Milwaukee 53226, USA
    Blood 101:1384-91. 2003
    ..Two amino acids, 416 in VWFpp and 869 in the mature VWF molecule, were identified as being critical for the association and granular storage of VWF...
  13. ncbi request reprint Re-establishment of VWF-dependent Weibel-Palade bodies in VWD endothelial cells
    Sandra L Haberichter
    Department of Pediatrics, Medical College of Wisconsin, Milwaukee 53226, USA
    Blood 105:145-52. 2005
    ..These VWF-null endothelial cells provide a unique opportunity to examine the biogenesis of Weibel-Palade bodies in endothelium from a canine model of type 3 VWD...
  14. ncbi request reprint von Willebrand factor storage and multimerization: 2 independent intracellular processes
    S L Haberichter
    Blood Research Institute, The Blood Center of Southeastern Wisconsin, Milwaukee, WI, USA
    Blood 96:1808-15. 2000
    ..vW AgII contains the signal(s) required for trafficking to storage, and only through interaction with vW AgII is vWF chaperoned into granules. (Blood. 2000;96:1808-1815)..
  15. ncbi request reprint Genetic mutations in von Willebrand disease identified by DHPLC and DNA sequence analysis
    Justin K Kakela
    BloodCenter of Wisconsin, 638 N 18th Street, P O Box 2178, Milwaukee, WI 53201, USA
    Mol Genet Metab 87:262-71. 2006
    ..This DHPLC and DNA sequencing technique will enable the full length assessment of the VWF gene necessary to detect mutations causing types 1 and 3 VWD...
  16. ncbi request reprint The von Willebrand factor propeptide (VWFpp) traffics an unrelated protein to storage
    Sandra L Haberichter
    Blood Research Institute, The Blood Center of Southeastern Wisconsin, Milwaukee, USA
    Arterioscler Thromb Vasc Biol 22:921-6. 2002
    ..We now demonstrate that VWFpp re-routes a constitutively secreted protein to the regulated storage pathway. Furthermore, our studies suggest that the VWFpp storage signal is contained within amino acids 201 to 741...
  17. ncbi request reprint The role of the D1 domain of the von Willebrand factor propeptide in multimerization of VWF
    Jonathan B Rosenberg
    Blood Research Institute, The Blood Center of Southeastern Wisconsin, Milwaukee, USA
    Blood 100:1699-706. 2002
    ..The integrity of the VWFpp is essential to maintain the proper spacing and alignment of the multiple cysteines in the VWFpp and N-terminus of the mature VWF...
  18. pmc Active tissue factor pathway inhibitor is expressed on the surface of coated platelets
    Susan A Maroney
    Blood Research Institute, Blood Center of Wisconsin, Milwaukee, WI 53201 2178, USA
    Blood 109:1931-7. 2007
    ..In association with its expression on the surface of coated platelets TFPI is also released in microvesicles or as a soluble protein...
  19. ncbi request reprint P-selectin binds to the D'-D3 domains of von Willebrand factor in Weibel-Palade bodies
    Gregoire Michaux
    MRC Laboratory for Molecular Cell Biology, Cell Biology Unit and Department of Biochemistry, University College London, Gower Street, London WC1E 6BT, United Kingdom
    Blood 107:3922-4. 2006
    ..Confirming the results of such a cellular assay by conventional coimmunoprecipitation, we concluded that the lumenal domain of P-selectin interacts with the D'-D3 domains of VWF...
  20. ncbi request reprint Factor VIII ectopically expressed in platelets: efficacy in hemophilia A treatment
    Helen V Yarovoi
    The Children s Hospital of Philadelphia, 1 Civic Center, ARC, Rm 317, Philadelphia, PA 19104, USA
    Blood 102:4006-13. 2003
    ..Our studies also suggest that platelet-released factor VIII is at least as potent as an equivalent plasma level and perhaps even more potent in an arterial thrombosis model...
  21. ncbi request reprint The physiological function of von Willebrand's factor depends on its tubular storage in endothelial Weibel-Palade bodies
    Gregoire Michaux
    Department of Biochemistry, University College London, Gower Street, London WC1E 6BT, United Kingdom
    Dev Cell 10:223-32. 2006
    ..Thus, a 100-fold compaction of VWF into tubules determines the unique shape of Weibel-Palade bodies and is critical to this protein's hemostatic function...