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Nutrients and Porphyria: An Intriguing Crosstalk

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...

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Detalles Bibliográficos
Autores principales: Di Pierro, Elena, Granata, Francesca
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7279006/
https://www.ncbi.nlm.nih.gov/pubmed/32422947
http://dx.doi.org/10.3390/ijms21103462
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author Di Pierro, Elena
Granata, Francesca
author_facet Di Pierro, Elena
Granata, Francesca
author_sort Di Pierro, Elena
collection PubMed
description 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.
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spelling pubmed-72790062020-06-15 Nutrients and Porphyria: An Intriguing Crosstalk Di Pierro, Elena Granata, Francesca Int J Mol Sci Review 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. MDPI 2020-05-14 /pmc/articles/PMC7279006/ /pubmed/32422947 http://dx.doi.org/10.3390/ijms21103462 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Di Pierro, Elena
Granata, Francesca
Nutrients and Porphyria: An Intriguing Crosstalk
title Nutrients and Porphyria: An Intriguing Crosstalk
title_full Nutrients and Porphyria: An Intriguing Crosstalk
title_fullStr Nutrients and Porphyria: An Intriguing Crosstalk
title_full_unstemmed Nutrients and Porphyria: An Intriguing Crosstalk
title_short Nutrients and Porphyria: An Intriguing Crosstalk
title_sort nutrients and porphyria: an intriguing crosstalk
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7279006/
https://www.ncbi.nlm.nih.gov/pubmed/32422947
http://dx.doi.org/10.3390/ijms21103462
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