Glutamine energy substrate anaplerosis increases bone density in the Pah(enu2) classical PKU mouse in the absence of phenylalanine restriction

Osteopenia is an under‐investigated clinical presentation of phenylalanine hydroxylase (PAH)‐deficient phenylketonuria (PKU). While osteopenia is not fully penetrant in human PKU, the Pah(enu2) mouse is universally osteopenic and ideal to study the phenotype. We determined Pah(enu2) mesenchymal stem...

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Detalles Bibliográficos
Autores principales: Dobrowolski, Steven F., Phua, Yu Leng, Tourkova, Irina L., Sudano, Cayla, Vockley, Jerry, Larrouture, Quitterie C., Blair, Harry C.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons, Inc. 2022
Materias:
Research Reports
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9458609/
https://www.ncbi.nlm.nih.gov/pubmed/36101821
http://dx.doi.org/10.1002/jmd2.12308

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9458609/
https://www.ncbi.nlm.nih.gov/pubmed/36101821
http://dx.doi.org/10.1002/jmd2.12308

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