Mesenchymal stem cell energy deficit and oxidative stress contribute to osteopenia in the Pah
Alkaline Phosphatase
/ genetics
Animals
Bone Density
/ genetics
Bone Diseases, Metabolic
/ complications
Cell Differentiation
/ drug effects
Disease Models, Animal
Humans
Mesenchymal Stem Cells
/ drug effects
Mice
Osteoblasts
/ drug effects
Oxidative Stress
/ genetics
Phenylalanine
/ genetics
Phenylalanine Hydroxylase
/ genetics
Phenylketonurias
/ complications
Resveratrol
/ pharmacology
Osteopenia
Oxidative stress
Pah(enu2)
Phenylketonuria
Respiratory complex 1
Journal
Molecular genetics and metabolism
ISSN: 1096-7206
Titre abrégé: Mol Genet Metab
Pays: United States
ID NLM: 9805456
Informations de publication
Date de publication:
03 2021
03 2021
Historique:
received:
16
12
2020
revised:
25
01
2021
accepted:
26
01
2021
pubmed:
20
2
2021
medline:
6
8
2021
entrez:
19
2
2021
Statut:
ppublish
Résumé
Osteopenia occurs in a subset of phenylalanine hydroxylase (PAH) deficient phenylketonuria (PKU) patients. While osteopenia is not fully penetrant in patients, the Pah
Identifiants
pubmed: 33602601
pii: S1096-7192(21)00034-2
doi: 10.1016/j.ymgme.2021.01.014
pmc: PMC9795491
mid: NIHMS1857184
pii:
doi:
Substances chimiques
Phenylalanine
47E5O17Y3R
Phenylalanine Hydroxylase
EC 1.14.16.1
Alkaline Phosphatase
EC 3.1.3.1
Resveratrol
Q369O8926L
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, U.S. Gov't, Non-P.H.S.
Langues
eng
Sous-ensembles de citation
IM
Pagination
173-179Subventions
Organisme : BLRD VA
ID : I01 BX002490
Pays : United States
Organisme : NIAMS NIH HHS
ID : R01 AR076146
Pays : United States
Organisme : NIDDK NIH HHS
ID : R01 DK109907
Pays : United States
Organisme : NIAMS NIH HHS
ID : R56 AR076146
Pays : United States
Informations de copyright
Copyright © 2021. Published by Elsevier Inc.
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