Unsuspected osteochondroma-like outgrowths in the cranial base of Hereditary Multiple Exostoses patients and modeling and treatment with a BMP antagonist in mice.

Sinha, Sayantani; Mundy, Christina; Bechtold, Till; Sgariglia, Federica; Ibrahim, Mazen M; Billings, Paul C; Carroll, Kristen; Koyama, Eiki; Jones, Kevin B; Pacifici, Maurizio

Sinha, Sayantani; Mundy, Christina; Bechtold, Till; Sgariglia, Federica; Ibrahim, Mazen M; Billings, Paul C; Carroll, Kristen; Koyama, Eiki; Jones, Kevin B; Pacifici, Maurizio.

Sinha S; Translational Research Program in Pediatric Orthopaedics, Division of Orthopaedic Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America.
Mundy C; Translational Research Program in Pediatric Orthopaedics, Division of Orthopaedic Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America.
Bechtold T; Translational Research Program in Pediatric Orthopaedics, Division of Orthopaedic Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America.
Sgariglia F; Translational Research Program in Pediatric Orthopaedics, Division of Orthopaedic Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America.
Ibrahim MM; Translational Research Program in Pediatric Orthopaedics, Division of Orthopaedic Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America.
Billings PC; Translational Research Program in Pediatric Orthopaedics, Division of Orthopaedic Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America.
Carroll K; Shriner's Hospital for Children, Salt Lake City, Utah, United States of America.
Koyama E; Department of Orthopaedics, University of Utah School of Medicine, Salt Lake City, Utah, United States of America.
Jones KB; Translational Research Program in Pediatric Orthopaedics, Division of Orthopaedic Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, United States of America.
Pacifici M; Department of Orthopaedics, University of Utah School of Medicine, Salt Lake City, Utah, United States of America.

PLoS Genet ; 13(4): e1006742, 2017 04.

Article en En | MEDLINE | ID: mdl-28445472

Asunto(s)

Exostosis Múltiple Hereditaria/genética; N-Acetilglucosaminiltransferasas/genética; Osteocondroma/genética; Proteína Smad1/genética; Animales; Proteínas Morfogenéticas Óseas/genética; Proteínas Morfogenéticas Óseas/metabolismo; Médula Cervical/metabolismo; Médula Cervical/patología; Condrogénesis/genética; Modelos Animales de Enfermedad; Desarrollo Embrionario/genética; Exostosis Múltiple Hereditaria/diagnóstico por imagen; Exostosis Múltiple Hereditaria/tratamiento farmacológico; Exostosis Múltiple Hereditaria/patología; Placa de Crecimiento/metabolismo; Placa de Crecimiento/patología; Heparitina Sulfato/biosíntesis; Humanos; Imagen por Resonancia Magnética; Ratones; Ratones Noqueados; Mutación; Osteocondroma/diagnóstico por imagen; Osteocondroma/patología; Pirazoles/administración & dosificación; Pirimidinas/administración & dosificación; Tomografía Computarizada de Emisión

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Texto completo: 1 Banco de datos: MEDLINE Asunto principal: Osteocondroma / Exostosis Múltiple Hereditaria / N-Acetilglucosaminiltransferasas / Proteína Smad1 Límite: Animals / Humans Idioma: En Año: 2017 Tipo del documento: Article

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