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Review
. 2020 May 14;21(10):3462.
doi: 10.3390/ijms21103462.

Nutrients and Porphyria: An Intriguing Crosstalk

Elena Di Pierro  1 , Francesca Granata  1
Affiliations
Review

Nutrients and Porphyria: An Intriguing Crosstalk

Elena Di Pierro et al. Int J Mol Sci. .

Abstract

Porphyria refers to a group of fascinating diseases from a metabolic and nutritional standpoint as it provides an example of how metabolic manipulation can be used for therapeutic purposes. It is characterized by defects in heme synthesis, particularly in the erythrocytes and liver. Specific enzymes involved in heme biosynthesis directly depend on adequate levels of vitamins and minerals in the tissues. Moreover, micronutrients that are required for producing succinyl CoA and other intermediates in the Krebs (TCA) cycle are indirectly necessary for heme metabolism. This review summarizes articles that describe the nutritional status, supplements intake, and dietary practices of patients affected by porphyria, paying special attention to the therapeutic use of nutrients that may help or hinder this group of diseases.

Keywords: diet; genetic factors; iron metabolism; lipid and glucose metabolism; nutrients; nutrigenomics; personalized nutrition; porphyria.

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Conflict of interest statement

The authors declare no conflicts of interest

Figures

Figure 1
Figure 1
Heme biosynthetic pathway, porphyrias and nutrients.
Figure 2
Figure 2
Regulation of ALAS-1 transcription by glucagon and insulin.
See this image and copyright information in PMC

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