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The chaperone-like sodium phenylbutyrate improves factor IX intracellular trafficking and activity impaired by the frequent p.R294Q mutation.
Pignani, S; Todaro, A; Ferrarese, M; Marchi, S; Lombardi, S; Balestra, D; Pinton, P; Bernardi, F; Pinotti, M; Branchini, A.
Afiliación
  • Pignani S; Department of Life Sciences and Biotechnology, University of Ferrara, Ferrara, Italy.
  • Todaro A; Department of Life Sciences and Biotechnology, University of Ferrara, Ferrara, Italy.
  • Ferrarese M; Department of Life Sciences and Biotechnology, University of Ferrara, Ferrara, Italy.
  • Marchi S; Department of Morphology, Surgery and Experimental Medicine, Section of Pathology, Oncology and Experimental Biology, University of Ferrara, Ferrara, Italy.
  • Lombardi S; Department of Life Sciences and Biotechnology, University of Ferrara, Ferrara, Italy.
  • Balestra D; Department of Life Sciences and Biotechnology, University of Ferrara, Ferrara, Italy.
  • Pinton P; Department of Morphology, Surgery and Experimental Medicine, Section of Pathology, Oncology and Experimental Biology, University of Ferrara, Ferrara, Italy.
  • Bernardi F; Department of Life Sciences and Biotechnology, University of Ferrara, Ferrara, Italy.
  • Pinotti M; Department of Life Sciences and Biotechnology, University of Ferrara, Ferrara, Italy.
  • Branchini A; Department of Life Sciences and Biotechnology, University of Ferrara, Ferrara, Italy.
J Thromb Haemost ; 16(10): 2035-2043, 2018 10.
Article en En | MEDLINE | ID: mdl-29993188
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Fenilbutiratos / Coagulación Sanguínea / Factor IX / Hemofilia B / Mutación Missense Tipo de estudio: Prognostic_studies Límite: Humans Idioma: En Revista: J Thromb Haemost Asunto de la revista: HEMATOLOGIA Año: 2018 Tipo del documento: Article País de afiliación: Italia
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Fenilbutiratos / Coagulación Sanguínea / Factor IX / Hemofilia B / Mutación Missense Tipo de estudio: Prognostic_studies Límite: Humans Idioma: En Revista: J Thromb Haemost Asunto de la revista: HEMATOLOGIA Año: 2018 Tipo del documento: Article País de afiliación: Italia