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4. Treatment with recombinant ADAMTS13, alleviates hypoxia/reoxygenation-induced pathologies in a mouse model of human sickle cell disease

Author

Rossato, P, Glantschnig, H, Canneva, F, Schuster, M, Coulibaly, S, Schrenk, G, Voelkel, D, Dockal, M, Plaimauer, B, Rottensteiner, H, Gritsch, H, Federti, E, Matte, A, DE FRANCESCHI, Lucia, Scheiflinger, F, and Hoellriegl, W

Subjects
mouse model, vaso-occlusive crisis, recombinant ADAMTS13, sickle cell disease, Hematology, ADAMTS13
Published
2023
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12. Evidence of protective effects of recombinant ADAMTS13 in a humanized model of sickle cell disease

Author

Rossato P., Federti E., Matte A., Glantschnig H., Canneva F., Schuster M., Coulibaly S., Schrenk G., Voelkel D., Dockal M., Plaimauer B., Andolfo I., Iolascon A., Rottensteiner H., Gritsch H., Scheiflinger F., Hoellriegl W., De Franceschi L., Rossato, P., Federti, E., Matte, A., Glantschnig, H., Canneva, F., Schuster, M., Coulibaly, S., Schrenk, G., Voelkel, D., Dockal, M., Plaimauer, B., Andolfo, I., Iolascon, A., Rottensteiner, H., Gritsch, H., Scheiflinger, F., Hoellriegl, W., and De Franceschi, L.

Subjects
congenital, hereditary, and neonatal diseases and abnormalities, ADAMTS13 Protein, Erythrocytes, Abnormal, inflammatory vasculopathy, Hematology, Anemia, Sickle Cell, Recombinant Proteins, Mice, Disease Models, Animal, Adamst13, hemic and lymphatic diseases, von Willebrand Factor, Animals, Humans, sickle cell disease, Vascular Diseases, Hypoxia
Abstract

Sickle cell disease (SCD) is an inherited red blood cell disorder that occurs worldwide. Acute vaso-occlusive crisis is the main cause of hospitalization in patients with SCD. There is growing evidence that inflammatory vasculopathy plays a key role in both acute and chronic SCD-related clinical manifestations. In a humanized mouse model of SCD, we found an increase of von Willebrand factor activity and a reduction in the ratio of a disintegrin and metalloproteinase with thrombospondin type 1 motif, number 13 (ADAMTS13) to von Willebrand factor activity similar to that observed in the human counterpart. Recombinant ADAMTS13 was administered to humanized SCD mice before they were subjected to hypoxia/reoxygenation (H/R) stress as a model of vaso-occlusive crisis. In SCD mice, recombinant ADAMTS13 reduced H/R-induced hemolysis and systemic and local inflammation in lungs and kidneys. It also diminished H/R-induced worsening of inflammatory vasculopathy, reducing local nitric oxidase synthase expression. Collectively, our data provide for the firsttime evidence that pharmacological treatment with recombinant ADAMTS13 (TAK-755) diminished H/R-induced sickle cell-related organ damage. Thus, recombinant ADAMTS13 might be considered as a potential effective disease-modifying treatment option for sickle cell-related acute events.

Published
2021

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