Heme metabolism: The janus nature of precursorsand porphyrins

Porphyrias comprise a group of metabolic disorders of the heme biosynthesis pathway resulting in a specific accumulation and excretion of intermediates which are responsible for their pathophysiology. Acute porphyrias are characterized by acute neurovisceral symptoms due to the overproduction and ac...

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Autores principales:	Rossetti, María Victoria, Buzaleh, Ana María, Parera, Victoria Estela, Fukuda, Haydeé, Lombardo, María Elisa, Lavandera, Jimena Verónica, Gerez, Esther Noemí, Diez, Berenice Andrea, Teijo, María Julieta, Guolo, Marcelo Nestor, Batlle, Alcira María del Carmen
Publicado:	2016
Materias:	D-aminolevulinic acid Heme metabolism Photodynamic therapy Porphyrias Porphyrins Trypanosoma cruzi Volatile anaesthetics
Acceso en línea:	https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_03252957_v50_n4_p547_Rossetti http://hdl.handle.net/20.500.12110/paper_03252957_v50_n4_p547_Rossetti
Aporte de:	Biblioteca Digital - Facultad de Ciencias Exactas y Naturales (UBA) de Universidad de Buenos Aires

id	paper:paper_03252957_v50_n4_p547_Rossetti
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spelling	paper:paper_03252957_v50_n4_p547_Rossetti2023-06-08T15:32:25Z Heme metabolism: The janus nature of precursorsand porphyrins Rossetti, María Victoria Buzaleh, Ana María Parera, Victoria Estela Fukuda, Haydeé Lombardo, María Elisa Lavandera, Jimena Verónica Gerez, Esther Noemí Diez, Berenice Andrea Teijo, María Julieta Guolo, Marcelo Nestor Batlle, Alcira María del Carmen D-aminolevulinic acid Heme metabolism Photodynamic therapy Porphyrias Porphyrins Trypanosoma cruzi Volatile anaesthetics Porphyrias comprise a group of metabolic disorders of the heme biosynthesis pathway resulting in a specific accumulation and excretion of intermediates which are responsible for their pathophysiology. Acute porphyrias are characterized by acute neurovisceral symptoms due to the overproduction and accumulation of d-aminolevulinic acid (ALA) which leads to an oxidative damage resulting from the formation of reactive oxygen species (ROS). In cutaneous porphyrias, the symptomatology is a result of porphyrin accumulation which also induces ROS moulding. In both cases, their clinical signs are precipitated by endogenous factors (stress, hormones, low calories intake) and/or exogenous drugs, in particular some anaesthetics. A review of the biochemical and genetic results obtained from patients with different porphyrias, diagnosed at the CIPYP during the last 38 years is presented here, aimed at obtaining additional evidence about the molecular nature of these disorders. The achievements obtained from experimental porphyria models -pharmacologically or genetically induced- are also described, which contributed to the classification of some drugs as prohibited for their use in porphyric patients. Finally, as porphyrins produce ROS and therefore cellular death, they can be used to treat infections by heme-deficient organisms like Trypanosoma cruzi and also as photosensitizers in photodynamic therapy (TFD). Fil:Rossetti, M.V. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. Fil:Buzaleh, A.M. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. Fil:Parera, V.E. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. Fil:Fukuda, H. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. Fil:Lombardo, M.E. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. Fil:Lavandera, J. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. Fil:Gerez, E.N. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. Fil:Diez, B.A. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. Fil:Teijo, M.J. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. Fil:Guolo, M.N. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. Fil:Del Carmen Batlle, A.M. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. 2016 https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_03252957_v50_n4_p547_Rossetti http://hdl.handle.net/20.500.12110/paper_03252957_v50_n4_p547_Rossetti
institution	Universidad de Buenos Aires
institution_str	I-28
repository_str	R-134
collection	Biblioteca Digital - Facultad de Ciencias Exactas y Naturales (UBA)
topic	D-aminolevulinic acid Heme metabolism Photodynamic therapy Porphyrias Porphyrins Trypanosoma cruzi Volatile anaesthetics
spellingShingle	D-aminolevulinic acid Heme metabolism Photodynamic therapy Porphyrias Porphyrins Trypanosoma cruzi Volatile anaesthetics Rossetti, María Victoria Buzaleh, Ana María Parera, Victoria Estela Fukuda, Haydeé Lombardo, María Elisa Lavandera, Jimena Verónica Gerez, Esther Noemí Diez, Berenice Andrea Teijo, María Julieta Guolo, Marcelo Nestor Batlle, Alcira María del Carmen Heme metabolism: The janus nature of precursorsand porphyrins
topic_facet	D-aminolevulinic acid Heme metabolism Photodynamic therapy Porphyrias Porphyrins Trypanosoma cruzi Volatile anaesthetics
description	Porphyrias comprise a group of metabolic disorders of the heme biosynthesis pathway resulting in a specific accumulation and excretion of intermediates which are responsible for their pathophysiology. Acute porphyrias are characterized by acute neurovisceral symptoms due to the overproduction and accumulation of d-aminolevulinic acid (ALA) which leads to an oxidative damage resulting from the formation of reactive oxygen species (ROS). In cutaneous porphyrias, the symptomatology is a result of porphyrin accumulation which also induces ROS moulding. In both cases, their clinical signs are precipitated by endogenous factors (stress, hormones, low calories intake) and/or exogenous drugs, in particular some anaesthetics. A review of the biochemical and genetic results obtained from patients with different porphyrias, diagnosed at the CIPYP during the last 38 years is presented here, aimed at obtaining additional evidence about the molecular nature of these disorders. The achievements obtained from experimental porphyria models -pharmacologically or genetically induced- are also described, which contributed to the classification of some drugs as prohibited for their use in porphyric patients. Finally, as porphyrins produce ROS and therefore cellular death, they can be used to treat infections by heme-deficient organisms like Trypanosoma cruzi and also as photosensitizers in photodynamic therapy (TFD).
author	Rossetti, María Victoria Buzaleh, Ana María Parera, Victoria Estela Fukuda, Haydeé Lombardo, María Elisa Lavandera, Jimena Verónica Gerez, Esther Noemí Diez, Berenice Andrea Teijo, María Julieta Guolo, Marcelo Nestor Batlle, Alcira María del Carmen
author_facet	Rossetti, María Victoria Buzaleh, Ana María Parera, Victoria Estela Fukuda, Haydeé Lombardo, María Elisa Lavandera, Jimena Verónica Gerez, Esther Noemí Diez, Berenice Andrea Teijo, María Julieta Guolo, Marcelo Nestor Batlle, Alcira María del Carmen
author_sort	Rossetti, María Victoria
title	Heme metabolism: The janus nature of precursorsand porphyrins
title_short	Heme metabolism: The janus nature of precursorsand porphyrins
title_full	Heme metabolism: The janus nature of precursorsand porphyrins
title_fullStr	Heme metabolism: The janus nature of precursorsand porphyrins
title_full_unstemmed	Heme metabolism: The janus nature of precursorsand porphyrins
title_sort	heme metabolism: the janus nature of precursorsand porphyrins
publishDate	2016
url	https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_03252957_v50_n4_p547_Rossetti http://hdl.handle.net/20.500.12110/paper_03252957_v50_n4_p547_Rossetti
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Heme metabolism: The janus nature of precursorsand porphyrins

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