Glutathione affects our ability to burn fat for energy

Did you know glutathione availability affects our ability to burn fat for energy?

This issue is a hallmark of our case - my older son doesn’t tolerate dietary fats (we don’t know yet which type) and doesn’t tolerate fasting either.

Once we figured fats are the problem, we did Acylcarnitine profile test and we also have Whole Genome Sequencing done for the whole family - this helps in investigation a bit.

The Acylcarnitine profile ruled out genetic fatty acid oxidation disorders, but it was indeed abnormal - with about 176% elevation for C18:2 from the end of the range.

But the overall profile is not consistent with any FAO disorder, so I started to suspect either he has more than one, or this is just an effect from nutritional deficiencies - it’s closer to MADD (Glutaric aciduria type 2), sort of Riboflavin deficiency or similar.

I checked all related FAO genes multiple times, and nothing stands out. So I agree with our metabolic doctor that it’s likely not a typical genetic FAO disorder. One evidence for that - 1-2 times a year my son does not experience any symptoms, has good cognition and almost like neurotypical - but it goes away quickly and we’re yet to find what causes this happy day. 🙂

Then I found that in rats, similar profile can be seen in Copper & Iron deficiency. Supplementing with iron (he was iron deficient) and copper (within RDA) does improve his state, but it’s still unstable.

And finally, found this article (R1):

“We wanted to know what happens to mitochondrial ability to burn fat with long-term glutathione deficiency in older people,” said Sekhar. “In fasted younger people, their body was able to burn fat well for energy needs. However, glutathione-deficient older people were not able to burn fat very well under the same conditions.”

“When we give these older people cysteine and glycine in their diets and corrected glutathione deficiency, amazingly their fat oxidation also normalized,” he said. “The older people go back to burning fat as well as younger people within 14 days.”