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Journal Articles

Factor VIII exhibits chaperone-dependent and glucose-regulated reversible amyloid formation in the endoplasmic reticulum

Juthakorn Poothong, Anita Pottekat, Marina Siirin, Alexandre Rosa Campos, Adrienne W. Paton, James C. Paton, Jacqueline Lagunas-Acosta, Zhouji Chen, Mark Swift, Niels Volkmann, Dorit Hanein, Jing Yong, Randal J. Kaufman

Blood (2020) 135 (21): 1899–1911.

https://doi.org/10.1182/blood.2019002867

Published: 2020

Includes: Supplemental data

Journal Articles

HRI coordinates translation by eIF2αP and mTORC1 to mitigate ineffective erythropoiesis in mice during iron deficiency

Shuping Zhang, Alejandra Macias-Garcia, Jason Velazquez, Elena Paltrinieri, Randal J. Kaufman, Jane-Jane Chen

Blood (2018) 131 (4): 450–461.

https://doi.org/10.1182/blood-2017-08-799908

Published: 2018

Includes: Supplemental data

Journal Articles

Molecular approaches for improved clotting factors for hemophilia

Randal J. Kaufman, Jerry S. Powell

Blood (2013) 122 (22): 3568–3574.

https://doi.org/10.1182/blood-2013-07-498261

Published: 2013

Journal Articles

Conservative mutations in the C2 domains of factor VIII and factor V alter phospholipid binding and cofactor activity

Gary E. Gilbert, Valerie A. Novakovic, Randal J. Kaufman, Hongzhi Miao, Steven W. Pipe

Blood (2012) 120 (9): 1923–1932.

https://doi.org/10.1182/blood-2012-01-408245

Published: 2012

Includes: Supplemental data

Journal Articles

Mice deficient in LMAN1 exhibit FV and FVIII deficiencies and liver accumulation of α1-antitrypsin

Bin Zhang, Chunlei Zheng, Min Zhu, Jiayi Tao, Matthew P. Vasievich, Andrea Baines, Jinoh Kim, Randy Schekman, Randal J. Kaufman, David Ginsburg

Blood (2011) 118 (12): 3384–3391.

https://doi.org/10.1182/blood-2011-05-352815

Published: 2011

Includes: Supplemental data

Journal Articles

In vivo efficacy of platelet-delivered, high specific activity factor VIII variants

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

Blood (2010) 116 (26): 6114–6122.

https://doi.org/10.1182/blood-2010-06-293308

Published: 2010

Includes: Supplemental data

Journal Articles

Endoplasmic Reticulum to Mitochondria Ca 2+ Signaling Inhibits Factor VIII Secretion and Mediates Oxidative Stress and Apoptosis Upon Factor VIII Expression

Jyoti Malhotra, Mon Xiong, Hongzhi Miao, Steven Pipe, Randal J. Kaufman, PhD

Blood (2010) 116 (21): 2212.

https://doi.org/10.1182/blood.V116.21.2212.2212

Published: 2010

Journal Articles

Factor VIII Biosynthesis and the Unfolded Protein Response.

Randal J. Kaufman, PhD

Blood (2009) 114 (22): SCI-19.

https://doi.org/10.1182/blood.V114.22.SCI-19.SCI-19

Published: 2009

Journal Articles

Incremental Improvement in Bioengineering of Coagulation Factor VIII for Efficient Expression: Elimination of a Dispensable Disulfide Loop Enhances Secretion

Sundar Rajan Selvaraj, PhD, Randal J Kaufman, PhD, Steven W. Pipe, MD

Blood (2008) 112 (11): 3073.

https://doi.org/10.1182/blood.V112.11.3073.3073

Published: 2008

Journal Articles

Exploiting ER Stress to Eliminate Inhibitor Antibodies in Hemophilia

Michael U Callaghan, MD, Randal J Kaufman, Ph.D.

Blood (2008) 112 (11): 3087.

https://doi.org/10.1182/blood.V112.11.3087.3087

Published: 2008

Journal Articles

Genotype-phenotype correlation in combined deficiency of factor V and factor VIII

Bin Zhang, Marta Spreafico, Chunlei Zheng, Angela Yang, Petra Platzer, Michael U. Callaghan, Zekai Avci, Namik Ozbek, Johnny Mahlangu, Tabitha Haw, Randal J. Kaufman, Kandice Marchant, Edward G. D. Tuddenham, Uri Seligsohn, Flora Peyvandi, David Ginsburg

Blood (2008) 111 (12): 5592–5600.

https://doi.org/10.1182/blood-2007-10-113951

Published: 2008

Includes: Supplemental data

Journal Articles

The Mutations SER2117→LEU, and TRP/TRP2063/64→MET/PHE in the Hydrophobic Spikes of the Factor V C2 Domain Lead to Tenacious Phospholipid Binding and Suggest a Mechanism through Which Phosphatidylserine Activates the Prothrombinase Complex.

Gary E. Gilbert, MD, Valerie A. Novakovic, M.S., Randal J. Kaufman, Ph.D., Hongzhi Miao, B.S., Steven W. Pipe, M.D.

Blood (2007) 110 (11): 2703.

https://doi.org/10.1182/blood.V110.11.2703.2703

Published: 2007

Journal Articles

Fatal hemorrhage in mice lacking γ-glutamyl carboxylase

Aihua Zhu, Hongmin Sun, Richard M. Raymond, Jr, Barbara C. Furie, Bruce Furie, Mila Bronstein, Randal J. Kaufman, Randal Westrick, David Ginsburg

Blood (2007) 109 (12): 5270–5275.

https://doi.org/10.1182/blood-2006-12-064188

Published: 2007

Journal Articles

Antioxidants Improve Factor VIII Secretion in the Liver by Preventing Oxidative Stress, Activation of the Unfolded Protein Response, and Apoptosis.

Jyoti D. Malhotra, PhD, Kezhong Zhang, Ph.D, Hongzi Miao, MS, Steven W. Pipe, MD, Randal J. Kaufman, Ph.D

Blood (2006) 108 (11): 197.

https://doi.org/10.1182/blood.V108.11.197.197

Published: 2006

Journal Articles

Inactivation-Resistant Recombinant Factor VIII Exhibits Superior Thrombin Generation Capacity in Comparison to Wild-Type and B Domain-Deleted Factor VIII.

Joshua Russell, Yesim Dargaud, MD, Randal J. Kaufman, PhD, Claude Negrier, MD, PhD, Steven W. Pipe, MD

Blood (2006) 108 (11): 1604.

https://doi.org/10.1182/blood.V108.11.1604.1604

Published: 2006

Journal Articles

Combined deficiency of factor V and factor VIII is due to mutations in either LMAN1 or MCFD2

Bin Zhang, Beth McGee, Jennifer S. Yamaoka, Hugo Guglielmone, Katharine A. Downes, Salvador Minoldo, Gustavo Jarchum, Flora Peyvandi, Norma B. de Bosch, Arlette Ruiz-Saez, Bernard Chatelain, Marian Olpinski, Paula Bockenstedt, Wolfgang Sperl, Randal J. Kaufman, William C. Nichols, Edward G. D. Tuddenham, David Ginsburg

Blood (2006) 107 (5): 1903–1907.

https://doi.org/10.1182/blood-2005-09-3620

Published: 2006

Journal Articles

Mutations in LMAN1 and MCFD2 May Account for All Cases of Combined Deficiency of Factor V and Factor VIII.

Bin Zhang, PhD, Beth McGee, BS, William C. Nichols, PhD, Hugo Guglielmone, M.Sc, Katherine Downes, MD, Flora Peyvandi, MD, Norma B. de Bosch, MD, Arlette Ruiz-Saez, MD, Bernard Chatelain, MD, Marian Olpinski, MD, Paula Bockenstedt, MD, Wolfgang Sperl, MD, Randal J. Kaufman, PhD, David Ginsburg, MD

Blood (2005) 106 (11): 724.

https://doi.org/10.1182/blood.V106.11.724.724

Published: 2005

Journal Articles

The Secretion Efficiency of Factor VIII Can Be Regulated by the Size and Oligosaccharide Content of the B Domain.

Steven W. Pipe, MD, Hongzhi Z. Miao, Philip F. Kucab, John H. McVey, PhD, Randal J. Kaufman, PhD

Blood (2005) 106 (11): 687.

https://doi.org/10.1182/blood.V106.11.687.687

Published: 2005

Journal Articles

Bioengineering of coagulation factor VIII for improved secretion

Hongzhi Z. Miao, Nongnuch Sirachainan, Lisa Palmer, Phillip Kucab, Michael A. Cunningham, Randal J. Kaufman, Steven W. Pipe

Blood (2004) 103 (9): 3412–3419.

https://doi.org/10.1182/blood-2003-10-3591

Published: 2004

Journal Articles

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

Steven W. Pipe, Evgueni L. Saenko, Angela N. Eickhorst, Geoffrey Kemball-Cook, Randal J. Kaufman

Blood (2001) 97 (3): 685–691.

https://doi.org/10.1182/blood.V97.3.685

Published: 2001

NARROW

Factor VIII exhibits chaperone-dependent and glucose-regulated reversible amyloid formation in the endoplasmic reticulum

HRI coordinates translation by eIF2αP and mTORC1 to mitigate ineffective erythropoiesis in mice during iron deficiency

Molecular approaches for improved clotting factors for hemophilia

Conservative mutations in the C2 domains of factor VIII and factor V alter phospholipid binding and cofactor activity

Mice deficient in LMAN1 exhibit FV and FVIII deficiencies and liver accumulation of α1-antitrypsin

In vivo efficacy of platelet-delivered, high specific activity factor VIII variants

Endoplasmic Reticulum to Mitochondria Ca 2+ Signaling Inhibits Factor VIII Secretion and Mediates Oxidative Stress and Apoptosis Upon Factor VIII Expression

Factor VIII Biosynthesis and the Unfolded Protein Response.

Incremental Improvement in Bioengineering of Coagulation Factor VIII for Efficient Expression: Elimination of a Dispensable Disulfide Loop Enhances Secretion

Exploiting ER Stress to Eliminate Inhibitor Antibodies in Hemophilia

Genotype-phenotype correlation in combined deficiency of factor V and factor VIII

The Mutations SER2117→LEU, and TRP/TRP2063/64→MET/PHE in the Hydrophobic Spikes of the Factor V C2 Domain Lead to Tenacious Phospholipid Binding and Suggest a Mechanism through Which Phosphatidylserine Activates the Prothrombinase Complex.

Fatal hemorrhage in mice lacking γ-glutamyl carboxylase

Antioxidants Improve Factor VIII Secretion in the Liver by Preventing Oxidative Stress, Activation of the Unfolded Protein Response, and Apoptosis.

Inactivation-Resistant Recombinant Factor VIII Exhibits Superior Thrombin Generation Capacity in Comparison to Wild-Type and B Domain-Deleted Factor VIII.

Combined deficiency of factor V and factor VIII is due to mutations in either LMAN1 or MCFD2

Mutations in LMAN1 and MCFD2 May Account for All Cases of Combined Deficiency of Factor V and Factor VIII.

The Secretion Efficiency of Factor VIII Can Be Regulated by the Size and Oligosaccharide Content of the B Domain.

Bioengineering of coagulation factor VIII for improved secretion

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

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American Society of Hematology

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American Society of Hematology

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