Antioxidant effects on the intracerebroventricular galactose damage in rats

Sasso, Simone; Cruz, Indianara Rodrigues; Lorenzini, Mariana Simonato; Magro, Débora Delwing‐Dal; Brueckheimer, Maitê Beatriz; Maia, Thayna Patachini; Sala, Geraldo Antonio Bunick Neto; Mews, Matheus Henrique Ruela; Lima, Daniela Delwing-de

doi:10.1016/j.prp.2018.07.034

Cited by 3 publications

(2 citation statements)

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“…Injection of galactose into rat cerebellums caused an increase in reactive oxygen species, damage to proteins and reduction in cognitive function. These effects were suppressed by the coadministration of the antioxidants ascorbic acid (vitamin C; CAS: 50-81-7; Figure 2) or α-tocopherol (vitamin E; CAS: 59-02-9; Figure 2) [35]. Ascorbate and the plant-derived xanthanoid α-mangostin (CAS: 6147-11-1; Figure 2) protected against oxidative stress and reduced the severity of the galactosemia-like phenotype in a Drosophila melanogaster model of the disease [38].…”

Section: Antioxidants -Dietarymentioning

confidence: 99%

“…The mechanism of this toxicity has not been definitively determined. It should also be noted that galactose itself is toxic at higher concentrations (>5 mM) and several studies have demonstrated that the administration of high levels of galactose to healthy animals results in similar pathology to galactosemia [34,35]. Disturbances in glycoprotein and glycolipid synthesis may partly explain the effects on the brain and ovaries [36,37].…”

Section: Introductionmentioning

confidence: 99%

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Repurposing drugs for the treatment of galactosemia

Timson

2019

Expert Opinion on Orphan Drugs

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Introduction: Galactosemia describes four diseases resulting from mutations in genes which code for enzymes involved in the metabolism of galactose and its derivatives. It has a wide range of symptoms ranging from the relatively mild (early onset cataracts) to severe damage to the liver, brain and ovaries which results in significant physical and cognitive disability. The only treatment is the removal or reduction of galactose in the diet. This treatment is unsatisfactory, particularly in the most severe forms of the disease. Considerable research efforts are being made to develop specific therapies for galactosemia. These include gene therapies, pharmacological chaperones, drugs to block the production of potentially toxic metabolites and enzyme replacement therapy. However, these are unlikely to be translated into the clinic for at least a decade.Areas covered: This review considers existing drugs, nutrients and treatments which could be relatively rapidly repurposed for the treatment of galactosemia. If successful, these would enable an improvement in the prognosis for galactosemia patients. Expert opinion: Dietary antioxidants which are already widely used and generally considered safe (e.g. resveratrol, purple sweet potato colour) should be tested for their efficacy in galactosemia.Pharmaceutical antioxidants (e.g. idebenone) should also be considered. Phosphate supplementation, along with careful monitoring of phosphate levels in the patient's diet should also be considered. Efforts to develop specific therapies for galactosemia should continue.

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Section: Antioxidants -Dietarymentioning

confidence: 99%