Ketogenic diet as a glycine lowering therapy in nonketotic hyperglycinemia and impact on brain glycine levels

Ketogenic diet as a glycine lowering therapy in nonketotic hyperglycinemia and impact on brain glycine levels

Abstract Background Nonketotic hyperglycinemia (NKH) is a severe neurometabolic disorder characterized by increased glycine levels. Current glycine reduction therapy uses high doses of sodium benzoate. The ketogenic diet (KD) may represent an alternative method of glycine reduction. Aim We aimed to...

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Bibliographic Details
Main Authors: Emily Shelkowitz, Russell P. Saneto, Walla Al-Hertani, Charlotte M. A. Lubout, Nicholas V. Stence, Mark S. Brown, Patrick Long, Diana Walleigh, Julie A. Nelson, Francisco E. Perez, Dennis W. W. Shaw, Emma J. Michl, Johan L. K. Van Hove
Format: Article
Language:English
Published: BMC 2022-12-01
Series:Orphanet Journal of Rare Diseases
Subjects:
Nonketotic hyperglycinemia
Benzoate
Ketogenic diet
Magnetic resonance spectroscopy
Glycine
Epilepsy
Online Access:https://doi.org/10.1186/s13023-022-02581-6

Internet

https://doi.org/10.1186/s13023-022-02581-6

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