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Report of two novel mutations in PTHLH associated with brachydactyly type E and literature review.
Thomas-Teinturier, Cecile; Pereda, Arrate; Garin, Intza; Diez-Lopez, Ignacio; Linglart, Agnès; Silve, Caroline; de Nanclares, Guiomar Pérez.
Afiliación
  • Thomas-Teinturier C; Pediatric Endocrinology, APHP, Hôpital Bicêtre, Le Kremlin Bicêtre, France.
  • Pereda A; Molecular (Epi)Genetics Laboratory, BioAraba National Health Institute, Hospital Universitario Araba-Txagorritxu, Vitoria-Gasteiz, Spain.
  • Garin I; Molecular (Epi)Genetics Laboratory, BioAraba National Health Institute, Hospital Universitario Araba-Txagorritxu, Vitoria-Gasteiz, Spain.
  • Diez-Lopez I; Department of Pediatrics, Hospital Universitario Araba-Txagorritxu, Vitoria-Gasteiz, Spain.
  • Linglart A; Pediatric Endocrinology, APHP, Hôpital Bicêtre, Le Kremlin Bicêtre, France.
  • Silve C; INSERM U1169, Hôpital Bicêtre, Le Kremlin Bicêtre, et Université Paris-Saclay, France.
  • de Nanclares GP; Centre de Référence des Maladies Rares du Métabolisme du calcium et du phosphore and Plateforme d'Expertise Paris Sud Maladies Rares, Hôpital Bicêtre, Le Kremlin Bicêtre, France.
Am J Med Genet A ; 170(3): 734-42, 2016 Mar.
Article en En | MEDLINE | ID: mdl-26640227
Autosomal-dominant brachydactyly type E is a congenital limb malformation characterized by small hands and feet as a result of shortened metacarpals and metatarsals. Alterations that predict haploinsufficiency of PTHLH, the gene coding for parathyroid hormone related protein (PTHrP), have been identified as a cause of this disorder in seven families. Here, we report three patients affected with brachydactyly type E, caused by PTHLH mutations expected to result in haploinsufficiency, and discuss our data compared to published reports.
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Texto completo: 1 Colección: 01-internacional Banco de datos: MEDLINE Asunto principal: Proteína Relacionada con la Hormona Paratiroidea / Braquidactilia / Mutación Tipo de estudio: Prognostic_studies / Risk_factors_studies Límite: Adult / Child / Female / Humans Idioma: En Revista: Am J Med Genet A Asunto de la revista: GENETICA MEDICA Año: 2016 Tipo del documento: Article País de afiliación: Francia

Texto completo: 1 Colección: 01-internacional Banco de datos: MEDLINE Asunto principal: Proteína Relacionada con la Hormona Paratiroidea / Braquidactilia / Mutación Tipo de estudio: Prognostic_studies / Risk_factors_studies Límite: Adult / Child / Female / Humans Idioma: En Revista: Am J Med Genet A Asunto de la revista: GENETICA MEDICA Año: 2016 Tipo del documento: Article País de afiliación: Francia