Harnessing the Power of Purple Sweet Potato Color and Myo-Inositol to Treat Classic Galactosemia
| Autor: | Synneva Hagen-Lillevik, Joshua Johnson, Anwer Siddiqi, Jes Persinger, Gillian Hale, Kent Lai |
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| Jazyk: | angličtina |
| Rok vydání: | 2022 |
| Předmět: | |
| Zdroj: | International Journal of Molecular Sciences, Vol 23, Iss 15, p 8654 (2022) |
| Druh dokumentu: | article |
| ISSN: | 23158654 1422-0067 1661-6596 |
| DOI: | 10.3390/ijms23158654 |
| Popis: | Classic Galactosemia (CG) is a devastating inborn error of the metabolism caused by mutations in the GALT gene encoding the enzyme galactose-1 phosphate uridylyltransferase in galactose metabolism. Severe complications of CG include neurological impairments, growth restriction, cognitive delays, and, for most females, primary ovarian insufficiency. The absence of the GALT enzyme leads to an accumulation of aberrant galactose metabolites, which are assumed to be responsible for the sequelae. There is no treatment besides the restriction of dietary galactose, which does not halt the development of the complications; thus, additional treatments are sorely needed. Supplements have been used in other inborn errors of metabolism but are not part of the therapeutic regimen for CG. The goal of this study was to test two generally recognized as safe supplements (purple sweet potato color (PSPC) and myo-inositol (MI)) that may impact cellular pathways contributing to the complications in CG. Our group uses a GalT gene-trapped mouse model to study the pathophysiology in CG, which phenocopy many of the complications. Here we report the ability of PSPC to ameliorate dysregulation in the ovary, brain, and liver of our mutant mice as well as positive results of MI supplementation in the ovary and brain. |
| Databáze: | Directory of Open Access Journals |
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