The Feature Paper can be either an original research article, a substantial novel research study that often involvesseveral techniques or approaches, or a comprehensive review paper with concise and precise updates on the latestprogress in the field that systematically reviews the most exciting advances in scientific literature. This type ofpaper provides an outlook on future directions of research or possible applications.
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The shape of the mean profiles for FXIII-A2B2 tetramer and total FXIII-A2 subunit corresponded to the FXIII activity profile. FXIII-A2 subunit and FXIII-A2B2 tetramer concentrations increased 4.5- and 7.6-fold, respectively, after rFXIII administration (Figure 1), then gradually declined during the subsequent month. The mean profile for uncomplexed B-subunit concentration was reversed (Figure 1) and decreased 4-fold after rFXIII injection. This was expected, because the FXIII-B subunit functions as a carrier protein for the FXIII-A2 subunit and, therefore, in the absence of FXIII-A2 subunit, there would be an excess of free FXIII-B subunit. Conversely, administration of rFXIII-A2 subunit results in rapid binding to free FXIII-B subunits and therefore decreases the concentration of free FXIII-B subunits.
Subunit concentrations. Mean SD concentration of the FXIII-A2 subunit (A) A2B2 tetramer (B), and B-subunit (C) are shown. FXIII-A2 subunit and FXIII-A2B2 tetramer concentrations increased after rFXIII administration. Because the FXIII-B subunit functions as a carrier protein for the FXIII-A2 subunit, administration of rFXIII resulted in decreased FXIII-B subunit because of the rapid binding of rFXIII to free FXIII-B subunit.
The present multicenter, multinational, open-label trial was undertaken to evaluate the efficacy and safety of this novel rFXIII in patients with congenital FXIII deficiency. The results confirm that prophylaxis with rFXIII is safe and effective for preventing bleeds in patients with congenital FXIII-A subunit deficiency.
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In the study, reported in Cell Discovery, the team found that both overall survival and progression-free survival (the time from surgery to relapse) were significantly longer in people who had high expression levels of ZNF667.
Faculty Detail Name X. LONG ZHENG Robert B. Adams Endowed Professor of PathologyDirector of Laboratory MedicineAttending Physician in Transfusion MedicineAssociate Editor, Journal of Thrombosis and HaemostasisSection Editor, Archives of Pathology and Laboratory Medicine Campus Address WP P230 Zip 7331 Phone (205) 975-8160 E-mail xzheng@uabmc.edu Other websites 1. UAB TTP Research Group2. TTP Foundation Award3. A possible novel therapy for TTP4. NIH grant supports TTP research 5. Dr. Zander Received ASH Congressional Fellowship6. Dr. McDaniel won the HTRS mentored award7. Dr. Zheng speaks at 9th BIC International Conference, Rome, Italy on TTP8. Dr. Zheng shares the Research Innovation in Scientific Excellence (RISE) Award from AABB9. A novel therapy for immune-mediated TTP10. Pathologist named NIH Study Section in Hemostasis11. Two blood clotting disorder with different causes interact synergistically
Research/Clinical Interest Title Molecular Mechanisms of Normal and Abnormal Blood Clotting; Inflammation and Microvascular Thrombosis; CRISPR/Cas9 Gene Editing as a Novel Therapeutic Strategy for Thrombotic Diseases Description Dr. Zheng's laboratory is interested in understanding the mechanisms of normal and abnormal blood coagulation, inflammation, and vascular biology. In particular, he and his colleagues focus on elucidating the pathogenesis of thrombotic thrombocytopenic purpura (TTP), a potentially fatal syndrome. Deficiency of plasma ADAMTS13 metalloprotease is the primary cause. ADAMTS13 is an enzyme that cleaves von Willebrand factor (VWF), an adhesion protein that helps agglutinate platelets for hemostasis. Deficiency of this enzyme leads to exaggerated platelet agglutination and the formation of microvascular thrombosis throughout the body, resulting in death. Current treatment is whole body plasma exchange. Dr. Zheng was among the first group of investigators who first discovered and cloned ADAMTS13 protease. In the past decade, Dr. Zheng's lab has made major contribution in determining the structure-function relationship and cofactor-dependent regulation of ADAMTS13. His lab is committed to develop novel tools for diagnosis and treatment of TTP. Potential rotation projects for graduate students include: a) Structure-function relationship of ADAMTS13; b) cofactor-dependent regulation of ADAMTS13 function; c) novel assay development for diagnosis of TTP; d) the role of systemic inflammation in pathogenesis of TTP; e) AAV-mediated gene therapy for hereditary and acquired TTP. Techniques in molecular biology, biochemistry/biophysics, and cell biology, as well as animal models are all commonly used in the laboratory. Current personnel: Wendy Cao, MD, PhD, Instructor; Vikram Pillai, PhD, Postdoctoral fellow; Liang Zheng, PhD, Postdoctoral fellow; Elizabeth M Staley, MD, PhD, Instructor; Mohammad Abdelgawwd, M.D., MS, graduate student, and Nicole Kocher, BS, research technician.
Selected Publications Publication PUBMEDID Cao W, Abdelgawwad MS, Li J, Zheng XL. Apolipoprotein B100/Low-Density Lipoprotein Regulates Proteolysis and Functions of von Willebrand Factor under Arterial Shear. Thromb Haemost. 2019 Sep 7. doi: 10.1055/s-0039-1696713. [Epub ahead of print] 31493779 Zheng L, Zhang D, Cao W, Song W, Zheng XL. Synergistic effects of ADAMTS13 deficiency and complement activation in pathogenesis of thrombotic microangiopathy. Blood. 2019 Aug 13. pii: blood.2019001040. doi: 10.1182/blood.2019001040. [Epub ahead of print] 31409673 Rottensteiner H, Seyfried BK, Kaufmann S, Fiedler C, Dong JF, Zheng XL, Plaimauer B, Scheiflinger F. Identification of cysteine thiol-based linkages in ADAMTS13 in support of a non-proteolytic regulation of von Willebrand factor. J Thromb Haemost. 2019 Aug 8. doi: 10.1111/jth.14602. [Epub ahead of print] 31393047 McDaniel JK, Abdelgawwad MS, Hargett A, Renfrow MB, Bdeir K, Cao W, Cines DB, Zheng XL. Human neutrophil peptide-1 inhibits thrombus formation under arterial flow via its terminal free cysteine thiols. J Thromb Haemost. 2019, 17(4):596-606. 30741476 Abdelgawwad M, Cao W, Zheng L, Kocher NK, Williams LA, and Zheng XL. Transfusion of Platelets Loaded With Recombinant ADAMTS13 (A Disintegrin and Metalloprotease With Thrombospondin Type 1 Repeats-13) Is Efficacious for Inhibiting Arterial Thrombosis Associated with Thrombotic Thrombocytopenic Puprura. Arterioscler Thromb Vasc Biol (ATVB). 2018 Nov;38(11):2731-2743. doi: 10.1161/ATVBAHA.118.311407. 30354235 Staley EM, Cao W, Pham HP, Kim CH, Kocher NK, Zheng L, Gangaraju R, Lorenz RG, Williams LA, Marques MB, Zheng XL. Clinical factors and biomarkers predicting outcome in patients with immune-mediated thrombotic thrombocytopenic purpura. Haematologica. 2018 Aug 31. pii: haematol.2018.198275. doi: 10.3324/haematol.2018.198275. 30171022 Kumar MA, Cao W, Pham HP, Raju D, Nawalinski K, Maloney-Wilensky E, Schuster J, and Zheng XL. Relative Deficiency of Plasma ADAMTS13 Activity and Elevation of Human Neutrophil Peptides in Patients with Traumatic Brain Injury. J Neurotruma. 2018 Aug 14. doi: 10.1089/neu.2018.5696. 29848170 Russell RT, McDaniel JK, Cao WJ, Shroyer M, Wagener BM, Zheng XL (corr. au.) and Pittet JF. Low Plasma ADAMTS13 Activity is Associated with Coagulopathy, Endothelial Cell Damage and Mortality after Severe Pediatric Trauma. Thromb Haemost. 2018 Apr;118(4):676-687. doi: 10.1055/s-0038-1636528. 29618154 Joly BS, Zheng XL and Veyradier A. Understanding thrombotic microangiopathies in children. Inten. Care Med., 2018 Sep;44(9):1536-1538. doi: 10.1007/s00134-018-5059-2. 29368057 Staley EM, Simmons SC, Feldman AZ, Lorenz RG, Marques MB, Williams LA 3rd, Zheng XL, Pham HP. Management of chronic myeloid leukemia in the setting of pregnancy: when is leukocytapheresis appropriate? A case report and review of the literature. Transfusion. 2018 Feb;58(2):456-460. doi: 10.1111/trf.14448. 29230832 Ping Z., A. Soni, L.A. Williams III, H.P. Pham, M.K. Base, and X.L. Zheng. Mutations in Coagulation Factor VIII Are Associated with More Favorable Outcome in Patients with Cutaneous Melanoma. TH Open 2017 Jul;1(2):e113-e121. 29152610 Xiao J, Feng Y, Li X, Li W, Fan L, Liu J, Zeng X, Chen K, Chen X, Zhou X, Zheng XL (corr. au.), and Chen S. Expression of ADAMTS13 in Normal and Abnormal Placentae and Its Potential Role in Angiogenesis and Placenta Development. Arterioscler Thromb Vasc Biol. (ATVB) 2017 Sep;37(9):1748-1756. 28751574 Saha M, McDanniel J, and Zheng XL. Pathogenesis, Diagnosis, And Potential Novel Therapeutics for Thrombotic Thrombocytopenic Purpurpa. J. Thromb. Haemost. 2017 Oct;15(10):1889-1900. 28662310 Kim CH, Simmons SC, Williams Iii LA, Staley EM, Zheng XL, Pham HP. ADAMTS13 test and/or PLASMIC clinical score in management of acquired thrombotic thrombocytopenic purpura: a cost-effective analysis. Transfusion. 2017 Nov;57(11):2609-2618. 28646526 Kumar M, Cao WJ, McDaniel JK, Pham HP, Raju D, Nawalinski K, Frangos S, Kung S, Zager E, Kasner SE, Levine JM, Zheng XL. Plasma ADAMTS13 activity and von Willebrand factor antigen and activity in patients with subarachnoid hemorrhage. Thromb. Haemost. 2017; 117(4):691-699. 28102428 Ueda Y, Mohammed I, Song D, Gullipalli D, Zhou L, Sato S, Wang Y, Gupta S, Cheng Z, Wang H, Bao J, Mao Y, Brass L, Zheng XL, Miwa T, Palmer M, Dunaief J, Song WC. Murine systemic thrombophilia and hemolytic uremic syndrome from a factor H point mutation. Blood. 2017; 129(9):1184-1196. 28057640 Nguyen GN, George LA, Siner JI, Davidson RJ, Zander CB, Zheng XL, Arruda VR, Camire RM, Sabatino DE. Novel factor VIII variants with a modified furin cleavage site improve the efficacy of gene therapy for hemophilia A. J Thromb Haemost. 2017;15(1):110-121. 27749002 Cao WJ, and Zheng XL. Conformational quiescence of ADAMTS13 prevents proteolytic promiscuity: comment. J Thromb Haemost. 2017;15(3):586-589. 28055145 Williams III LA, Drwiega JC, Cao W, Pham HP, Bertoli LF, and Zheng XL. Acquired autoimmune thrombotic thrombocytopenic purpura in a case of severe hemophilia A. Haemophilia. 2016; 22(6):e565-e567. 27704646 Zheng L, Mao Y, Abdelgawwad MS, Kocher NK, Li M, Dai X, Li B, and Zheng XL. Therapeutic Efficacy of Platelet Glycoprotein Ib Antagonist Anfibatide in Murine Models of Thrombotic Thrombocytopenia Purpura. Blood Adv. 2016 1:75-83. 28480350 Cao W, Pham HP, Williams LA, McDaniel J, Siniard RC, Lorenz RG, Marques MB, Zheng XL. Human Neutrophil Peptides And Complement Factor Bb In Pathogenesis Of Acquired Thrombotic Thrombocytopenic Purpura. Haematologica. 2016; 101(11):1319-1326. 27662014 Pillar V.G., Bao J., Zander C.B., McDaniel J.K., Chetty P.S., Seeholzer S.H., Bdeir K., Cines D.B., Zheng X.L. Human neutrophil peptides inhibit cleavage of von Willebrand factor by ADAMTS13: a potential link of inflammation to TTP. Blood. 2016;28(1):110-9. 27207796 Ostertag EM, Kacir S, Thiboutot M, Gulendran G, Zheng XL, Cines DB, Siegel DL. ADAMTS13 autoantibodies cloned from patients with acquired thrombotic thrombocytopenic purpura: 1. Structural and functional characterization in vitro. Transfusion. 2016;56(7):1763-74. 27040144 Ostertag EM, Bdeir K, Kacir S, Thiboutot M, Gulendran G, Yunk L, Hayes VM, Motto DG, Poncz M, Zheng XL, Cines DB, Siegel DL. ADAMTS13 autoantibodies cloned from patients with acquired thrombotic thrombocytopenic purpura: 2. Pathogenicity in an animal model. Transfusion. 2016;56(7):1775-85. 27040023 Tutwiler V, Madeeva D, Ahn HS, Andrianova I, Hayes V, Zheng XL, Cines DB, McKenzie SE, Poncz M, Rauova L. Platelet transactivation by monocytes promotes the prothrombotic state in heparin-induced thrombocytopenia. Blood. 2016;127(4):464-72 26518435 Casina VC, Hu W, Mao JH, Lu RN, Hanby HA, Picken B, Kan ZY, Lim WK, Mayne L, Ostertag EM, Kacir S, Siegel DL, Englander SW, and Zheng XL. High resolution epitope mapping by HX MS reveals the pathogenic mechanism and a possible therapy for autoimmune TTP syndrome. Proc Natl Acad Sci USA. 2015;112:9620-5. 26203127 Zander CB, Cao W, Zheng XL. ADAMTS13 and von Willebrand factor interaction. Curr Opin Hematol. 2015;22:452-9. 26186678 Zheng XL. ADAMTS13 and von Willebrand Factor in Thrombotic Thrombocytopenic Purpura. Annu Rev Med. 2015; 66: 211-25. 25587650 Pickens B, Mao Y, Li D, Siegel DL, Poncz M, Cines DB, and Zheng XL. Platelet-delivered ADAMTS13 inhibits arterial thrombosis and prevents murine models of thrombotic thrombocytopenic purpura. Blood. 2015;125 (21):3326-34. 25800050 Zheng XL. ADAMTS13, lucky to have a hydrophobic pocket. Blood. 2015;125 (12):1852-3. 25792731 Lu RN, Yang S, Wu HM, Zheng XL. Unconjugated Bilirubin Inhibits Proteolytic Cleavage of von Willebrand Factor by ADAMTS13 Protease. J Thromb Haemost. 2015;13(6):1064-72. 25782102 Bauer RC, Tohyama J, Cui J, Cheng L, Yang J, Zhang X, Ou K, Paschos GK, Zheng XL, Parmacek MS, Rader DJ, Reilly MP. Knockout of Adamts7, A Novel CAD Locus in Humans, Reduces Atherosclerosis in Mice. Circulation. 2015;131(13):1202-13. 25712206 Zheng XL. DNase, ADAMTS13, and iPAD4: good for the heart. Blood. 2014;123:10-1. 24385497 Grillberger R, Casina VC, Turecek PL, Zheng XL, Rottensteiner H, Scheiflinger F. Anti-ADAMTS13 IgG autoantibodies present in healthy individuals share linear epitopes with those in patients with thrombotic thrombocytopenic purpura. Haematologica. 2014;99:e58-60. 24532042 Chen J, Zhou H, Diacovo A, Zheng XL, Emsley J, Diacovo TG. Exploiting the kinetic interplay between GPIb-VWF binding interfaces to regulate hemostasis and thrombosis. Blood. 2014;124:3799-807. 25293780 Bao J, Xiao J, Mao Y, Zheng XL. Carboxyl terminus of ADAMTS13 directly inhibits platelet aggregation and ultra large von Willebrand factor string formation under flow in a free-thiol-dependent manner. Arterioscler Thromb Vasc Biol. (ATVB) 2014;34:397-407. 24357063 Skipwith CG, Haberichter SL, Gehrand A, Zheng XL. Compromised shear-dependent cleavage of type 2N von Willebrand factor variants by ADAMTS13 in the presence of factor VIII. Thromb Haemost. 2013;110:202-4. 23636243 Jin SY, Xiao J, Bao J, Zhou S, Wright JF, Zheng XL. AAV-mediated expression of an ADAMTS13 variant prevents shigatoxin-induced thrombotic thrombocytopenic purpura. Blood. 2013;121:3825-9, 23515928 Cao W, Sabatino DE, Altynova E, Lange AM, Casina VC, Camire RM, Zheng XL. Light chain of factor VIII is sufficient for accelerating cleavage of von Willebrand factor by ADAMTS13 metalloprotease. J Biol Chem. 2012;287:32459-66 22854959 Jin SY, Tohyama J, Bauer RC, Cao NN, Rader DJ, Zheng XL. Genetic ablation of Adamts13 gene dramatically accelerates the formation of early atherosclerosis in a murine model. Arterioscler Thromb Vasc Biol. (ATVB) 2012;32:1817-23. 22652598 Zheng XL. ADAMTS13 meets MR, then what? Blood. 2012;119:3652-4. 22517873 Wroblewska A, van Haren SD, Herczenik E, Kaijen P, Ruminska A, Jin SY, Zheng XL, van den Biggelaar M, ten Brinke A, Meijer AB, Voorberg J. Modification of an exposed loop in the C1 domain reduces immune responses to factor VIII in hemophilia A mice. Blood. 2012;119:5294-300. 22498747 Jian C, Xiao J, Gong L, Skipwith CG, Jin SY, Kwaan HC, Zheng XL. Gain-of-function ADAMTS13 variants that are resistant to autoantibodies against ADAMTS13 in patients with acquired thrombotic thrombocytopenic purpura. Blood. 2012;119:3836-43. 22289888 Xiao J, Jin SY, Xue J, Sorvillo N, Voorberg J, Zheng XL. Essential domains of a disintegrin and metalloprotease with thrombospondin type 1 repeats-13 metalloprotease required for modulation of arterial thrombosis. Arterioscler Thromb Vasc Biol. (ATVB) 2011;31:2261-9. 21799176 Jobes D, Wolfe Y, O'Neill D, Calder J, Jones L, Sesok-Pizzini D, Zheng XL. Toward a definition of "fresh" whole blood: an in vitro characterization of coagulation properties in refrigerated whole blood for transfusion. Transfusion. 2011;51:43-51. 20663116 Skipwith CG, Cao W, Zheng XL. Factor VIII and platelets synergistically accelerate cleavage of von Willebrand factor by ADAMTS13 under fluid shear stress. J Biol Chem. 2010;285:28596-603. 20605782 Zheng XL, Wu HM, Shang D, Falls E, Skipwith CG, Cataland SR, Bennett CL, Kwaan HC. Multiple domains of ADAMTS13 are targeted by autoantibodies against ADAMTS13 in patients with acquired idiopathic thrombotic thrombocytopenic purpura. Haematologica. 2010;95:1555-62. 20378566 Jin SY, Skipwith CG, Zheng XL. Amino acid residues Arg(659), Arg(660), and Tyr(661) in the spacer domain of ADAMTS13 are critical for cleavage of von Willebrand factor. Blood. 2010;115:2300-10. 20075158 Raife TJ, Cao W, Atkinson BS, Bedell B, Montgomery RR, Lentz SR, Johnson GF, Zheng XL. Leukocyte proteases cleave von Willebrand factor at or near the ADAMTS13 cleavage site. Blood. 2009;114:1666-74. 19541819 Laje P, Shang D, Cao W, Niiya M, Endo M, Radu A, DeRogatis N, Scheiflinger F, Zoltick PW, Flake AW, Zheng XL. Correction of murine ADAMTS13 deficiency by hematopoietic progenitor cell-mediated gene therapy. Blood. 2009;113:2172-80. 19141866 2ff7e9595c
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