Research Notes by Sergey
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May 3, 2023
  • #Nrf2
  • #Cystine
  • #Persulfides
  • #xCT
  • #SLC7A11
  • #SQOR

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References

Elevated ethylmalonic acid can be a result of Nrf2 activation

If I read this right, then elevated ethylmalonic acid can be a result of Nrf2 activation.

Because Cystine that the cell take up during upregulated xCT transporter will undergo processing inside mitochondria and result in thiosulfate and sulfite production.

Here, we demonstrated that NRF2 increased intracellular persulfides by upregulating the cystine transporter xCT encoded by Slc7a11, a well-known NRF2 target gene. Persulfides have been shown to play an important role in mitochondrial function.

Supplementation with glutathione trisulfide (GSSSG), which is a form of persulfide, elevated the mitochondrial membrane potential (MMP), increased the oxygen consumption rate (OCR) and promoted ATP production.

Persulfide-mediated mitochondrial activation was shown to require the mitochondrial sulfur oxidation pathway, especially sulfide quinone oxidoreductase (SQOR).

Consistently, NRF2-mediated mitochondrial activation was also dependent on SQOR activity.

References

1
Contribution of NRF2 to sulfur metabolism and mitochondrial activity
2023
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