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The onset of PI3K-related vascular malformations occurs during angiogenesis and is prevented by the AKT inhibitor miransertib

Kobialka, Piotr (author)
Josep Carreras Leukaemia Res Inst, Endothelial Pathobiol & Microenvironm, Barcelona, Spain.
Sabata, Helena (author)
Josep Carreras Leukaemia Res Inst, Endothelial Pathobiol & Microenvironm, Barcelona, Spain.
Vilalta, Odena (author)
Josep Carreras Leukaemia Res Inst, Endothelial Pathobiol & Microenvironm, Barcelona, Spain.
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Gouveia, Leonor (author)
Uppsala universitet,Vaskulärbiologi,Josep Carreras Leukaemia Res Inst, Endothelial Pathobiol & Microenvironm, Barcelona, Spain
Angulo-Urarte, Ana (author)
Josep Carreras Leukaemia Res Inst, Endothelial Pathobiol & Microenvironm, Barcelona, Spain.
Muixi, Laia (author)
Josep Carreras Leukaemia Res Inst, Endothelial Pathobiol & Microenvironm, Barcelona, Spain.
Zanoncello, Jasmina (author)
Josep Carreras Leukaemia Res Inst, Endothelial Pathobiol & Microenvironm, Barcelona, Spain.
Muñoz-Aznar, Oscar (author)
Inst Recerca St Joan de Deu, Dev Tumor Biol Lab, Barcelona, Spain.
Olaciregui, Nagore G. (author)
Inst Recerca St Joan de Deu, Dev Tumor Biol Lab, Barcelona, Spain.
Fanlo, Lucia (author)
Josep Carreras Leukaemia Res Inst, 3D Chromatin Org, Barcelona, Spain.
Esteve-Codina, Anna (author)
Barcelona Inst Sci & Technol, Ctr Genom Regulat, CNAG CRG, Barcelona, Spain.;Univ Pompeu Fabra UPF, Barcelona, Spain.
Lavarino, Cinzia (author)
Inst Recerca St Joan de Deu, Dev Tumor Biol Lab, Barcelona, Spain.
Javierre, Biola M. (author)
Josep Carreras Leukaemia Res Inst, 3D Chromatin Org, Barcelona, Spain.
Celis, Veronica (author)
Hosp St Joan de Deu Barcelona, Pediat Canc Ctr Barcelona, Barcelona, Spain.
Rovira, Carlota (author)
Hosp St Joan de Deu Barcelona, Dept Pathol, Barcelona, Spain.
López-Fernández, Susana (author)
Hosp Santa Creu & Sant Pau, Dept Plast Surg, Barcelona, Spain.
Baselga, Eulàlia (author)
Hosp St Joan de Deu, Dept Dermatol, Barcelona, Spain.
Mora, Jaume (author)
Inst Recerca St Joan de Deu, Dev Tumor Biol Lab, Barcelona, Spain.;Hosp St Joan de Deu Barcelona, Pediat Canc Ctr Barcelona, Barcelona, Spain.
Castillo, Sandra D. (author)
Josep Carreras Leukaemia Res Inst, Endothelial Pathobiol & Microenvironm, Barcelona, Spain.
Graupera, Mariona (author)
Josep Carreras Leukaemia Res Inst, Endothelial Pathobiol & Microenvironm, Barcelona, Spain.;Inst Salud Carlos III, CIBERONC, Madrid, Spain.
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Josep Carreras Leukaemia Res Inst, Endothelial Pathobiol & Microenvironm, Barcelona, Spain Vaskulärbiologi (creator_code:org_t)
2022-06-13
2022
English.
In: EMBO Molecular Medicine. - : EMBO Press. - 1757-4676 .- 1757-4684. ; 14:7
Related links:
https://doi.org/10.1...
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https://uu.diva-port... (primary) (Raw object)
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https://doi.org/10.1...
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  • Journal article (peer-reviewed)
Abstract Subject headings
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  • Low-flow vascular malformations are congenital overgrowths composed of abnormal blood vessels potentially causing pain, bleeding and obstruction of different organs. These diseases are caused by oncogenic mutations in the endothelium, which result in overactivation of the PI3K/AKT pathway. Lack of robust in vivo preclinical data has prevented the development and translation into clinical trials of specific molecular therapies for these diseases. Here, we demonstrate that the Pik3caH1047R activating mutation in endothelial cells triggers a transcriptome rewiring that leads to enhanced cell proliferation. We describe a new reproducible preclinical in vivo model of PI3K-driven vascular malformations using the postnatal mouse retina. We show that active angiogenesis is required for the pathogenesis of vascular malformations caused by activating Pik3ca mutations. Using this model, we demonstrate that the AKT inhibitor miransertib both prevents and induces the regression of PI3K-driven vascular malformations. We confirmed the efficacy of miransertib in isolated human endothelial cells with genotypes spanning most of human low-flow vascular malformations.

Subject headings

MEDICIN OCH HÄLSOVETENSKAP  -- Medicinska och farmaceutiska grundvetenskaper -- Cell- och molekylärbiologi (hsv//swe)
MEDICAL AND HEALTH SCIENCES  -- Basic Medicine -- Cell and Molecular Biology (hsv//eng)

Keyword

AKT
angiogenesis
endothelial cell
PI3K
vascular malformations

Publication and Content Type

ref (subject category)
art (subject category)

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