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Haploinsufficient TNAP Mice Display Decreased Extracellular ATP Levels and Expression of Pannexin-1 Channels

Hypophosphatasia (HPP) is a rare heritable metabolic bone disease caused by hypomorphic mutations in the ALPL (in human) or Akp2 (in mouse) gene, encoding the tissue-nonspecific alkaline phosphatase (TNAP) enzyme. In addition to skeletal and dental malformations, severe forms of HPP are also charact...

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Detalles Bibliográficos
Autores principales: Sebastián-Serrano, Álvaro, de Diego-García, Laura, Henshall, David C., Engel, Tobías, Díaz-Hernández, Miguel
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5841270/
https://www.ncbi.nlm.nih.gov/pubmed/29551976
http://dx.doi.org/10.3389/fphar.2018.00170