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Steven Pipe

Research Profile

Chemicals & Drugs

Disorders

Hemophilia A

Procedures

Protein Engineering

Living Beings

Physiology

Concepts & Ideas

Protein Structure, Tertiary

Anatomy

Phenomena

Protein Binding

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Publications

TOP 3
Gary E Gilbert; Randal J Kaufman; Hongzhi Miao; Valerie A Novakovic; Steven W Pipe
Conservative mutations in the C2 domains of factor VIII and factor V alter phospholipid binding and cofactor activity.
Brian W Gray; Robert A Drongowski; Raquel Gonzalez; Steven W Pipe; Lauren A Stephens; Kavita S Warrier; George B Mychaliska
Characterization of central venous catheter-associated deep venous thrombosis in infants.
Steven W Pipe
The hope and reality of long-acting hemophilia products.

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All Publications

2012: Gary E Gilbert; Randal J Kaufman; Hongzhi Miao; Valerie A Novakovic; Steven W Pipe
Conservative mutations in the C2 domains of factor VIII and factor V alter phospholipid binding and cofactor activity.
Blood 2012;120(9):1923-32.
2012: Brian W Gray; Robert A Drongowski; Raquel Gonzalez; Steven W Pipe; Lauren A Stephens; Kavita S Warrier; George B Mychaliska
Characterization of central venous catheter-associated deep venous thrombosis in infants.
Journal of pediatric surgery 2012;47(6):1159-66.
2012: Steven W Pipe
The hope and reality of long-acting hemophilia products.
American journal of hematology 2012;87 Suppl 1():S33-9.
2012: L A Valentino; Y Xiong; X Ye; Steven W Pipe; M D Tarantino; M P Luo
Healthcare resource utilization among haemophilia A patients in the United States.
Haemophilia : the official journal of the World Federation of Hemophilia 2012;18(3):332-8.
2012: Neil A Goldenberg; R Kruse-Jarres; Cindy A Leissinger; Steven W Pipe; N Frick; C M Kessler
Outcomes of mentored, grant-funded fellowship training in haemostasis /thrombosis: findings from a nested case-control survey study.
Haemophilia : the official journal of the World Federation of Hemophilia 2012;18(3):326-31.
2012: B Sørensen; Y Dargaud; G Kenet; Jeanne M Lusher; Andrew D Mumford; Steven W Pipe; A Tiede
On-demand treatment of bleeds in haemophilia patients with inhibitors: strategies for securing and maintaining predictable efficacy with recombinant activated factor VII.
Haemophilia : the official journal of the World Federation of Hemophilia 2012;18(2):255-62.
2012: S R Selvaraj; A N Scheller; Randal J Kaufman; H Z Miao; Steven W Pipe
Bioengineering of coagulation factor VIII for efficient expression through elimination of a dispensable disulfide loop.
Journal of thrombosis and haemostasis : JTH 2012;10(1):107-15.
2011: Steven W Pipe; Stephen P Butler; J Calcaterra; H Miao; William H Velander
Functional factor VIII made with von Willebrand factor at high levels in transgenic milk.
Journal of thrombosis and haemostasis : JTH 2011;9(11):2235-42.
2011: K Ogata; H Z Miao; S R Selvaraj; Steven W Pipe
Most factor VIII B domain missense mutations are unlikely to be causative mutations for severe hemophilia A: implications for genotyping.
Journal of thrombosis and haemostasis : JTH 2011;9(6):1183-90.
2011: Junhong Lü; Steven W Pipe; Hongzhi Miao; Marc Jacquemin; Gary E Gilbert
A membrane-interactive surface on the factor VIII C1 domain cooperates with the C2 domain for cofactor function.
Blood 2011;117(11):3181-9.
2011: Steven W Pipe; Barbara Zieger
Factors for life: advances in the treatment of congenital and coagulopathic bleeding disorders.
Thrombosis research 2011;128 Suppl 1():S1.
2010: Teshell K Greene; Cheng Wang; Jessica D Hirsch; Li Zhai; Jamie Gewirtz; Michael A Thornton; Hongzhi Z Miao; Steven W Pipe; Randal J Kaufman; Rodney M Camire; Valder R Arruda; M Anna Kowalska; Mortimer Poncz
In vivo efficacy of platelet-delivered, high specific activity factor VIII variants.
Blood 2010;116(26):6114-22.
2010: Steven W Pipe
Go long! A touchdown for factor VIII?
Blood 2010;116(2):153-4.
2010: Jeanne M Lusher; Steven W Pipe; S Alexander; D Nugent
Prophylactic therapy with Fibrogammin P is associated with a decreased incidence of bleeding episodes: a retrospective study.
Haemophilia : the official journal of the World Federation of Hemophilia 2010;16(2):316-21.
2010: Steven W Pipe
Hemophilia: new protein therapeutics.
Hematology / the Education Program of the American Society of Hematology. American Society of Hematology. Education Program 2010;2010():203-9.
2010: Rui-Jun Su; Angela Epp; Yvette Latchman; Doug Bolgiano; Steven W Pipe; Neil C Josephson
Suppression of FVIII inhibitor formation in hemophilic mice by delivery of transgene modified apoptotic fibroblasts.
Molecular therapy : the journal of the American Society of Gene Therapy 2010;18(1):214-22.
2009: Steven W Pipe
Visions in haemophilia care.
Thrombosis research 2009;124 Suppl 2():S2-5.
2009: Steven W Pipe
Functional roles of the factor VIII B domain.
Haemophilia : the official journal of the World Federation of Hemophilia 2009;15(6):1187-96.
2009: Anjali Alatkar Sharathkumar; Nancy Lamear; Steven W Pipe; Sanjay Parikh; Mark Russell; Abrahim Simon; Amy Shapiro
Management of neonatal aortic arch thrombosis with low-molecular weight heparin: a case series.
Journal of pediatric hematology/oncology 2009;31(7):516-21.
2009: L A Valentino; Michael Recht; J Dipaola; A D Shapiro; Steven W Pipe; N Ewing; J Urgo; T Bullock; M Simmons; C Deguzman
Experience with a third generation recombinant factor VIII concentrate (Advate) for immune tolerance induction in patients with haemophilia A.
Haemophilia : the official journal of the World Federation of Hemophilia 2009;15(3):718-26.
2009: Steven W Pipe
Antihemophilic factor (recombinant) plasma/albumin-free method for the management and prevention of bleeding episodes in patients with hemophilia A.
Biologics : targets & therapy 2009;3():117-25.
2009: A Cuker; B Ptashkin; B A Konkle; Steven W Pipe; Herbert C Whinna; X L Zheng; D B Cines; E S Pollak
Interlaboratory agreement in the monitoring of unfractionated heparin using the anti-factor Xa-correlated activated partial thromboplastin time.
Journal of thrombosis and haemostasis : JTH 2009;7(1):80-6.
2008: Jyoti D Malhotra; Hongzhi Miao; Kezhong Zhang; Anna Wolfson; Subramaniam Pennathur; Steven W Pipe; Randal J Kaufman
Antioxidants reduce endoplasmic reticulum stress and improve protein secretion.
Proceedings of the National Academy of Sciences of the United States of America 2008;105(47):18525-30.
2008: S Kasuda; A Kubo; Yoshihiko Sakurai; S Irion; K Ohashi; K Tatsumi; Y Nakajima; Y Saito; K Hatake; Steven W Pipe; Midori Shima; Akira Yoshioka
Establishment of embryonic stem cells secreting human factor VIII for cell-based treatment of hemophilia A.
Journal of thrombosis and haemostasis : JTH 2008;6(8):1352-9.
2008: R d'Oiron; Steven W Pipe; M Jacquemin
Mild/moderate haemophilia A: new insights into molecular mechanisms and inhibitor development.
Haemophilia : the official journal of the World Federation of Hemophilia 2008;14 Suppl 3():138-46.
2008: Steven W Pipe; K A High; K Ohashi; A. Ugur URAL; D Lillicrap
Progress in the molecular biology of inherited bleeding disorders.
Haemophilia : the official journal of the World Federation of Hemophilia 2008;14 Suppl 3():130-7.
2008: Jialan Shi; Steven W Pipe; Jan T Rasmussen; Christian W Heegaard; Gary E Gilbert
Lactadherin blocks thrombosis and hemostasis in vivo: correlation with platelet phosphatidylserine exposure.
Journal of thrombosis and haemostasis : JTH 2008;6(7):1167-74.
2008: Steven W Pipe
Recombinant clotting factors.
Thrombosis and haemostasis 2008;99(5):840-50.
2008: Anjali Sharathkumar; Steven W Pipe
Post-thrombotic syndrome in children: a single center experience.
Journal of pediatric hematology/oncology 2008;30(4):261-6.
2008: Anjali A Sharathkumar; Steven W Pipe
Bleeding disorders.
Pediatrics in review / American Academy of Pediatrics 2008;29(4):121-29; quiz 130.
2007: Vincenzo Cerullo; Michael Seiler; Viraj Mane; Racel Cela; Christian Clarke; Randal J Kaufman; Steven W Pipe; Brendan Lee
Correction of murine hemophilia A and immunological differences of factor VIII variants delivered by helper-dependent adenoviral vectors.
Molecular therapy : the journal of the American Society of Gene Therapy 2007;15(12):2080-7.
2007: Steven W Pipe; L A Valentino
Optimizing outcomes for patients with severe haemophilia A.
Haemophilia : the official journal of the World Federation of Hemophilia 2007;13 Suppl 4():1-16; quiz 3 p following 16.
2007: A Sharathkumar; Ronald B Hirschl; Steven W Pipe; C Crandell; Barbara S Adams; J J Lin
Primary thromboprophylaxis with heparins for arteriovenous fistula failure in pediatric patients.
The journal of vascular access 2007;8(4):235-44.
2007: Lingxia Chen; Fuxiang Zhu; Juan Li; Hui Lu; Haiyan Jiang; Rita Sarkar; Valder R Arruda; Jinhui Wang; Jennifer Zhao; Glenn F Pierce; Qiulan Ding; Xuefeng Wang; Hongli Wang; Steven W Pipe; Xiang-Qin Liu; Xiao Xiao; Rodney M Camire; Weidong Xiao
The enhancing effects of the light chain on heavy chain secretion in split delivery of factor VIII gene.
Molecular therapy : the journal of the American Society of Gene Therapy 2007;15(10):1856-62.
2007: Anjali Alatkar Sharathkumar; Christina Crandall; Jen-Jar Lin; Steven W Pipe
Treatment of thrombosis with fondaparinux (Arixtra) in a patient with end-stage renal disease receiving hemodialysis therapy.
Journal of pediatric hematology/oncology 2007;29(8):581-4.
2007: Donna DiMichele; W Keith Hoots; Steven W Pipe; Georges-Etienne Rivard; E Santagostino
International workshop on immune tolerance induction: consensus recommendations.
Haemophilia : the official journal of the World Federation of Hemophilia 2007;13 Suppl 1():1-22.
2007: Glenn F Pierce; David Lillicrap; Steven W Pipe; Thierry VandenDriessche
Gene therapy, bioengineered clotting factors and novel technologies for hemophilia treatment.
Journal of thrombosis and haemostasis : JTH 2007;5(5):901-6.
2007: Nicole Dowling; M G Beckman; Marilyn J Manco-Johnson; K Hassell; Claire S Philipp; Lisa A Michaels; Stephan Moll; John A Heit; John A Penner; Roshni Kulkarni; Steven W Pipe; Paula L Bockenstedt; Judith C Andersen; S Crudder; Andra H James; S Zimmerman; Thomas L Ortel
The U.S. Thrombosis and Hemostasis Centers pilot sites program.
Journal of thrombosis and thrombolysis 2007;23(1):1-7.
2006: Evgueni L Saenko; Steven W Pipe
Strategies towards a longer acting factor VIII.
Haemophilia : the official journal of the World Federation of Hemophilia 2006;12 Suppl 3():42-51.
2006: Courtney Thornburg; Steven W Pipe
Neonatal thromboembolic emergencies.
Seminars in fetal & neonatal medicine 2006;11(3):198-206.
2006: Steven W Pipe
Consideration in hemophilia therapy selection.
Seminars in hematology 2006;43(2 Suppl 3):S23-7.
2006: Courtney D Thornburg; Steven W Pipe
Adherence to prophylactic infusions of factor VIII or factor IX for haemophilia.
Haemophilia : the official journal of the World Federation of Hemophilia 2006;12(2):198-9.
2006: Steven W Pipe
The physician's role in selecting a factor replacement therapy.
Haemophilia : the official journal of the World Federation of Hemophilia 2006;12 Suppl 1():21-5; discussion 26-8.
2005: Steven W Pipe
The promise and challenges of bioengineered recombinant clotting factors.
Journal of thrombosis and haemostasis : JTH 2005;3(8):1692-701.
2005: Nongnuch Sirachainan; Bin Zhang; Ampaiwan Chuansumrit; Steven W Pipe; Werasak Sasanakul; David Ginsburg
Combined factor V and factor VIII deficiency in a Thai patient: a case report of genotype and phenotype characteristics.
Haemophilia : the official journal of the World Federation of Hemophilia 2005;11(3):280-4.
2004: Steven W Pipe; J-M Saint-Remy; Christopher E Walsh
New high-technology products for the treatment of haemophilia.
Haemophilia : the official journal of the World Federation of Hemophilia 2004;10 Suppl 4():55-63.
2004: Hongzhi Z Miao; Nongnuch Sirachainan; Lisa Palmer; Phillip Kucab; Michael A Cunningham; Randal J Kaufman; Steven W Pipe
Bioengineering of coagulation factor VIII for improved secretion.
Blood 2004;103(9):3412-9.
2004: Steven W Pipe
Coagulation factors with improved properties for hemophilia gene therapy.
Seminars in thrombosis and hemostasis 2004;30(2):227-37.
2003: Michael A Cunningham; Steven W Pipe; Bin Zhang; Hans-Peter Hauri; David Ginsburg; Randal J Kaufman
LMAN1 is a molecular chaperone for the secretion of coagulation factor VIII.
Journal of thrombosis and haemostasis : JTH 2003;1(11):2360-7.
2003: Tony L Yang; Steven W Pipe; Angela Yang; David Ginsburg
Biosynthetic origin and functional significance of murine platelet factor V.
Blood 2003;102(8):2851-5.
2003: Courtney D Thornburg; Keri A Lattimore; Steven W Pipe
Tricuspid valve thrombus and pulmonary embolus in an infant with homozygous thermolabile methylenetetrahydrofolate reductase and heterozygous prothrombin G20210A variant.
Journal of perinatology : official journal of the California Perinatal Association 2003;23(6):513-5.
2003: Bin Zhang; Michael A Cunningham; William C Nichols; John A Bernat; Uri Seligsohn; Steven W Pipe; John H McVey; Ursula Schulte-Overberg; Norma de Bosch; Arlette Ruiz-Saez; Gilbert C White; Edward G D Tuddenham; Randal J Kaufman; David Ginsburg
Bleeding due to disruption of a cargo-specific ER-to-Golgi transport complex.
Nature genetics 2003;34(2):220-5.
2003: Evgueni L Saenko; Natalya M Ananyeva; Midori Shima; Charlotte A E Hauser; Steven W Pipe
The future of recombinant coagulation factors.
Journal of thrombosis and haemostasis : JTH 2003;1(5):922-30.
2002: William H Hakeos; Hongzhi Miao; Nongnuch Sirachainan; Geoffrey Kemball-Cook; Evgueni L Saenko; Randal J Kaufman; Steven W Pipe
Hemophilia A mutations within the factor VIII A2-A3 subunit interface destabilize factor VIIIa and cause one-stage/two-stage activity discrepancy.
Thrombosis and haemostasis 2002;88(5):781-7.
2002: Evgueni L Saenko; Natalya M Ananyeva; Diana Kouiavskaia; Horst Schwinn; Djuro Josic; Midori Shima; Charlotte A E Hauser; Steven W Pipe
Molecular defects in coagulation Factor VIII and their impact on Factor VIII function.
Vox sanguinis 2002;83(2):89-96.
2002: Serguei Soukharev; David Hammond; Natalya M Ananyeva; Julia A M Anderson; Charlotte A E Hauser; Steven W Pipe; Evgueni L Saenko
Expression of factor VIII in recombinant and transgenic systems.
Blood cells, molecules & diseases 2002;28(2):234-48.
2002: Jennifer L Grow; Paola J Fliman; Steven W Pipe
Neonatal sinovenous thrombosis associated with homozygous thermolabile methylenetetrahydrofolate reductase in both mother and infant.
Journal of perinatology : official journal of the California Perinatal Association 2002;22(2):175-8.
2002: Gary E Gilbert; Randal J Kaufman; Andrew A Arena; Hongzhi Miao; Steven W Pipe
Four hydrophobic amino acids of the factor VIII C2 domain are constituents of both the membrane-binding and von Willebrand factor-binding motifs.
The Journal of biological chemistry 2002;277(8):6374-81.
2001: EJ Renaud; D Liu; Steven W Pipe; M A Rubin; Daniel H Teitelbaum
Inferior vena cavectomy for nonexcisable Wilms' tumor thrombus.
Journal of pediatric surgery 2001;36(3):526-9.
2001: Steven W Pipe; Evgueni L Saenko; A N Eickhorst; Geoffrey Kemball-Cook; Randal J Kaufman
Hemophilia A mutations associated with 1-stage/2-stage activity discrepancy disrupt protein-protein interactions within the triplicated A domains of thrombin-activated factor VIIIa.
Blood 2001;97(3):685-91.
2000: Steven W Pipe; Randal J Kaufman
A chamber of hope for hemophilia.
Nature biotechnology 2000;18(3):264-5.
1999: MJ Moussalli; Steven W Pipe; Hans-Peter Hauri; William C Nichols; David Ginsburg; Randal J Kaufman
Mannose-dependent endoplasmic reticulum (ER)-Golgi intermediate compartment-53-mediated ER to Golgi trafficking of coagulation factors V and VIII.
The Journal of biological chemistry 1999;274(46):32539-42.
1999: Randal J Kaufman; Steven W Pipe
Regulation of factor VIII expression and activity by von Willebrand factor.
Thrombosis and haemostasis 1999;82(2):201-8.
1999: Steven W Pipe; A N Eickhorst; S H McKinley; Evgueni L Saenko; Randal J Kaufman
Mild hemophilia A caused by increased rate of factor VIII A2 subunit dissociation: evidence for nonproteolytic inactivation of factor VIIIa in vivo.
Blood 1999;93(1):176-83.
1998: Randal J Kaufman; Steven W Pipe
Can we improve on nature? "Super molecules" of factor VIII.
Haemophilia : the official journal of the World Federation of Hemophilia 1998;4(4):370-9.
1998: Steven W Pipe; J A Morris; J Shah; Randal J Kaufman
Differential interaction of coagulation factor VIII and factor V with protein chaperones calnexin and calreticulin.
The Journal of biological chemistry 1998;273(14):8537-44.
1997: Randal J Kaufman; Steven W Pipe; Luigina Tagliavacca; M Swaroop; MJ Moussalli
Biosynthesis, assembly and secretion of coagulation factor VIII.
Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis 1997;8 Suppl 2():S3-14.
1997: Steven W Pipe; Randal J Kaufman
Characterization of a genetically engineered inactivation-resistant coagulation factor VIIIa.
Proceedings of the National Academy of Sciences of the United States of America 1997;94(22):11851-6.
1997: M Swaroop; MJ Moussalli; Steven W Pipe; Randal J Kaufman
Mutagenesis of a potential immunoglobulin-binding protein-binding site enhances secretion of coagulation factor VIII.
The Journal of biological chemistry 1997;272(39):24121-4.
1996: Steven W Pipe; Randal J Kaufman
Factor VIII C2 domain missense mutations exhibit defective trafficking of biologically functional proteins.
The Journal of biological chemistry 1996;271(41):25671-6.
1996: Steven W Pipe; Alvin H Schmaier; William C Nichols; David Ginsburg; Mary Ellen A Hernandez; Valerie P Castle
Neonatal purpura fulminans in association with factor V R506Q mutation.
The Journal of pediatrics 1996;128(5 Pt 1):706-9.
1993: T Trus; Andrea Winthrop; Steven W Pipe; J Shah; Jacob C Langer; G Y Lau
Optimal management of patent ductus arteriosus in the neonate weighing less than 800 g.
Journal of pediatric surgery 1993;28(9):1137-9.