Search Results - "Scandella, Dorothea"

The Acidic Region of the Factor VIII Light Chain and the C2 Domain Together Form the High Affinity Binding Site for von Willebrand Factor by Saenko, Evgueni L., Scandella, Dorothea

“…A binding site for von Willebrand factor (vWf) was previously localized to the carboxyl terminus of the C2 domain of the light chain (LCh) of factor VIII…”
Get full text

Long-term induction of immune tolerance after blockade of CD40-CD40L interaction in a mouse model of hemophilia A by Rossi, Gabriela, Sarkar, Jolly, Scandella, Dorothea

“…A factor VIII–deficient knockout mouse was used as a model for severe hemophilia A to characterize the immune response to recombinant human factor VIII (fVIII)…”
Get full text

Human Inhibitor Antibodies Specific for the Factor VIII A2 Domain Disrupt the Interaction between the Subunit and Factor IXa by Fay, Philip J., Scandella, Dorothea

“…Factor VIIIa, a heterotrimer of the A1, A2, and A3-C1-C2 subunits, increases the catalytic efficiency for factor IXa-catalyzed activation of factor X. A…”
Get full text

Slowed Release of Thrombin-cleaved Factor VIII from von Willebrand Factor by a Monoclonal and a Human Antibody Is a Novel Mechanism for Factor VIII Inhibition by Saenko, Evgueni L., Shima, Midori, Gilbert, Gary E., Scandella, Dorothea

“…The anti-factor VIII (fVIII) C2 domain monoclonal antibody ESH8 inhibits fVIII activity only when fVIII is bound to von Willebrand factor (vWf). However, ESH8…”
Get full text

A Mechanism for Inhibition of Factor VIII Binding to Phospholipid by von Willebrand Factor by Saenko, Evgueni L., Scandella, Dorothea

“…von Willebrand factor (vWf) acts as a carrier for blood coagulation factor VIII (fVIII) in the circulation. The amino-terminal 272 residues of mature vWf…”
Get full text

Role of Factor VIII C2 Domain in Factor VIII Binding to Factor Xa by Nogami, Keiji, Shima, Midori, Hosokawa, Kazuya, Suzuki, Toyoaki, Koide, Takehiko, Saenko, Evgueni L., Scandella, Dorothea, Shibata, Masaru, Kamisue, Seiki, Tanaka, Ichiro, Yoshioka, Akira

“…Factor VIII (FVIII) is activated by proteolytic cleavages with thrombin and factor Xa (FXa) in the intrinsic blood coagulation pathway. The anti-C2 monoclonal…”
Get full text

Activation of Factor VIII by Thrombin Increases Its Affinity for Binding to Synthetic Phospholipid Membranes and Activated Platelets by Saenko, Evgueni L., Scandella, Dorothea, Yakhyaev, Alexey V., Greco, Nicholas J.

“…Membrane-bound thrombin-activated factor VIII (fVIIIa) functions as a cofactor for factor IXa in the factor Xase complex. We found that binding of…”
Get full text

Transgenic pigs produce functional human factor VIII in milk by Paleyanda, Rekha K, Velander, William H, Lee, Timothy K, Scandella, Dorothea H, Gwazdauskas, Francis C, Knight, James W, Hoyer, Leon W, Drohan, William N, Lubon, Henryk

“…Deficiency or abnormality of coagulation factor VIII (FVIII) causes a bleeding disorder called hemophilia A. Treatment involves FVIII concentrates prepared…”
Get full text

In Hemophilia A and Autoantibody Inhibitor Patients: The Factor VIII A2 Domain and Light Chain Are Most Immunogenic by Scandella, Dorothea H., Nakai, Hiroaki, Felch, Matthew, Mondorf, Wolfgang, Scharrer, Inge, Hoyer, Leon W., Saenko, Evgueni L.

“…Factor VIII (fVIII) is a protein cofactor essential for blood coagulation, and it binds in the factor Xase complex to factors IXa, X, and phospholipid. In…”
Get full text

Residues 484-508 Contain a Major Determinant of the Inhibitory Epitope in the A2 Domain of Human Factor VIII by Healey, John F., Lubin, Ira M., Nakai, Hiroaki, Saenko, Evgueni L., Hoyer, Leon W., Scandella, Dorothea, Lollar, Pete

“…The A2 domain (residues 373-740) of human blood coagulation factor VIII (fVIII) contains a major epitope for inhibitory alloantibodies and autoantibodies. We…”
Get full text

A factor VIII neutralizing monoclonal antibody and a human inhibitor alloantibody recognizing epitopes in the C2 domain inhibit factor VIII binding to von Willebrand factor and to phosphatidylserine by Shima, M, Scandella, D, Yoshioka, A, Nakai, H, Tanaka, I, Kamisue, S, Terada, S, Fukui, H

“…A neutralizing monoclonal antibody, NMC-VIII/5, recognizing the 72 kDa thrombin-proteolytic fragment of factor VIII light chain was obtained. Binding of the…”
Get more information

Analysis of the Human Factor VIII A2 Inhibitor Epitope by Alanine Scanning Mutagenesis by Lubin, Ira M., Healey, John F., Barrow, Rachel T., Scandella, Dorothea, Lollar, Pete

“…Antibodies directed to the A2 domain of factor VIII (fVIII) are usually an important component of the polyclonal response in patients who have clinically…”
Get full text

Common inhibitory effects of human anti-C2 domain inhibitor alloantibodies on factor VIII binding to von Willebrand factor by Shima, M, Nakai, H, Scandella, D, Tanaka, I, Sawamoto, Y, Kamisue, S, Morichika, S, Murakami, T, Yoshioka, A

“…Factor VIII (FVIII) inhibitor alloantibodies obtained from seven severe haemophilia A patients were examined for their binding regions and their effects on…”
Get more information

Dominant C2 domain epitope specificity of inhibitor antibodies elicited by a heat pasteurized product, factor VIII CPS-P, in previously treated hemophilia A patients without inhibitors by Sawamoto, Y, Prescott, R, Zhong, D, Saenko, E L, Mauser-Bunschoten, E, Peerlinck, K, van den Berg, M, Scandella, D

“…From June, 1990, to November, 1991, in The Netherlands and Belgium, 16 previously treated severe hemophilia A patients (PTP) developed inhibitors after…”
Get more information

An alloantibody recognizing the FVIII A1 domain in a patient with CRM reduced haemophilia A due to deletion of a large portion of the A1 domain DNA sequence by Shibata, M, Shima, M, Morichika, S, McVey, J, Tuddenham, E G, Tanaka, I, Suzuki, H, Nogami, K, Minamoto, Y, Hato, T, Saenko, E L, Scandella, D, Yoshioka, A

“…We report the development of a FVIII inhibitor in a patient with severe, cross reacting material reduced (CRM(R)) haemophilia A. The level of Factor VIII…”
Get more information

Identification of a factor VIII peptide, residues 2315–2330, which neutralizes human factor VIII C2 inhibitor alloantibodies: requirement of Cys2326 and Glu2327 for maximum effect by Nogami, Keiji, Shima, Midori, Nakai, Hiroaki, Tanaka, Ichiro, Suzuki, Hiroshi, Morichika, Shogo, Shibata, Masaru, Saenko, Evgueni, Scandella, Dorothea, Giddings, John, Yoshioka, Akira

“…Factor VIII (FVIII) inhibitor alloantibodies react with combinations of the A2, C2 and A3‐C1 domains of the FVIII molecule. Some inhibitors block binding of…”
Get full text

New characteristics of anti-factor VIII inhibitor antibody epitopes and unusual immune responses to Factor VIII by Scandella, Dorothea

“…Treatment of individuals with severe hemophilia A by plasma-derived or recombinant factor VIII leads to the production of anti-factor VIII antibodies in…”
Get more information

Anti-factor VIII inhibitor alloantibodies recognizing the A2 domain in the human factor VIII heavy chain poorly bind to porcine factor VIII by Sawamoto, Y, Shima, M, Tanaka, I, Nakai, H, Kamisue, S, Scandella, D, Yoshioka, A

“…Anti-factor VIII (FVIII) inhibitor alloantibodies from 11 patients with hemophilia A, along with five anti-FVIII neutralizing monoclonal antibodies, were…”
Get more information

Reduction of the antigenicity of factor VIII toward complex inhibitory antibody plasmas using multiply-substituted hybrid human/porcine factor VIII molecules by BARROW, R. T, HEALEY, J. F, GAILANI, D, SCANDELLA, D, LOLLAR, P

“…Factor VIII (fVIII) circulates as a heavy chain/light chain (A1-A2-B/ap-A3-C1-C2) heterodimer. The 41-residue light chain activation peptide, ap, is cleaved…”
Get full text

Epitope Specificity and Inactivation Mechanisms of Factor VIII Inhibitor Antibodies by Scandella, Dorothea

“…The domain specificity of anti‐factor VIII (FVIII) inhibitor antibodies was determined in assays using FVIII domains generated by thrombin cleavage or…”
Get full text