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The chaperone activity of 4PBA ameliorates the skeletal phenotype of Chihuahua, a zebrafish model for dominant osteogenesis imperfecta.
Gioia, Roberta; Tonelli, Francesca; Ceppi, Ilaria; Biggiogera, Marco; Leikin, Sergey; Fisher, Shannon; Tenedini, Elena; Yorgan, Timur A; Schinke, Thorsten; Tian, Kun; Schwartz, Jean-Marc; Forte, Fabiana; Wagener, Raimund; Villani, Simona; Rossi, Antonio; Forlino, Antonella.
Affiliation
  • Gioia R; Department of Molecular Medicine, Biochemistry Unit, University of Pavia, Pavia, Italy.
  • Tonelli F; Department of Molecular Medicine, Biochemistry Unit, University of Pavia, Pavia, Italy.
  • Ceppi I; Department of Molecular Medicine, Biochemistry Unit, University of Pavia, Pavia, Italy.
  • Biggiogera M; Department of Biology and Biotechnology, University of Pavia, Pavia, Italy.
  • Leikin S; Section on Physical Biochemistry, Eunice Kennedy Shriver NICHD, NIH, Bethesda, MD, USA.
  • Fisher S; Department of Pharmacology & Experimental Therapeutics, Boston University School of Medicine, Boston, MA, USA.
  • Tenedini E; Center for Genome Research, Department of Medical and Surgical Sciences, University of Modena and Reggio Emilia, Modena, Italy.
  • Yorgan TA; Institute of Osteology and Biomechanic, Center for Experimental Medicine, University of Hamburg, Hamburg, Germany.
  • Schinke T; Institute of Osteology and Biomechanic, Center for Experimental Medicine, University of Hamburg, Hamburg, Germany.
  • Tian K; Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK.
  • Schwartz JM; Faculty of Biology, Medicine and Health, University of Manchester, Manchester, UK.
  • Forte F; Medical Faculty, Center for Biochemistry, Center for Molecular Medicine, University of Cologne, Cologne, Germany.
  • Wagener R; Medical Faculty, Center for Biochemistry, Center for Molecular Medicine, University of Cologne, Cologne, Germany.
  • Villani S; Department of Public Health and Experimental and Forensic Medicine, Unit of Biostatistics and Clinical Epidemiology, University of Pavia, Pavia, Italy.
  • Rossi A; Department of Molecular Medicine, Biochemistry Unit, University of Pavia, Pavia, Italy.
  • Forlino A; Department of Molecular Medicine, Biochemistry Unit, University of Pavia, Pavia, Italy.
Hum Mol Genet ; 26(15): 2897-2911, 2017 08 01.
Article in En | MEDLINE | ID: mdl-28475764
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Osteogenesis Imperfecta / Phenylbutyrates Limits: Animals Language: En Journal: Hum Mol Genet Journal subject: BIOLOGIA MOLECULAR / GENETICA MEDICA Year: 2017 Document type: Article Affiliation country: Italy Country of publication: United kingdom
Fulltext
Add to My VHL
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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Osteogenesis Imperfecta / Phenylbutyrates Limits: Animals Language: En Journal: Hum Mol Genet Journal subject: BIOLOGIA MOLECULAR / GENETICA MEDICA Year: 2017 Document type: Article Affiliation country: Italy Country of publication: United kingdom