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, M.V., Buzaleh, A.M., Parera, V.E., Fukuda, H., Lombardo, M.E., Lavandera, J., Gerez, E.N., Melito, V.A., Zuccoli, J.R., Ruspini, S.F., Puente, V.R., Diez, B.A., Teijo, M.J., Cerbino, G.N., Varela, L.S., Guolo, M.N., Del Carmen Batlle, A.M.
Formato: JOUR
Materias:
D-aminolevulinic acid
Heme metabolism
Photodynamic therapy
Porphyrias
Porphyrins
Trypanosoma cruzi
Volatile anaesthetics
Acceso en línea:http://hdl.handle.net/20.500.12110/paper_03252957_v50_n4_p547_Rossetti
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Biblioteca Digital - Facultad de Ciencias Exactas y Naturales (UBA) de Universidad de Buenos Aires
id todo:paper_03252957_v50_n4_p547_Rossetti
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spelling todo:paper_03252957_v50_n4_p547_Rossetti2023-10-03T15:23:44Z Heme metabolism: The janus nature of precursorsand porphyrins Rossetti, M.V. Buzaleh, A.M. Parera, V.E. Fukuda, H. Lombardo, M.E. Lavandera, J. Gerez, E.N. Melito, V.A. Zuccoli, J.R. Ruspini, S.F. Puente, V.R. Diez, B.A. Teijo, M.J. Cerbino, G.N. Varela, L.S. Guolo, M.N. Del Carmen Batlle, A.M. 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. JOUR info:eu-repo/semantics/openAccess http://creativecommons.org/licenses/by/2.5/ar 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, M.V.
Buzaleh, A.M.
Parera, V.E.
Fukuda, H.
Lombardo, M.E.
Lavandera, J.
Gerez, E.N.
Melito, V.A.
Zuccoli, J.R.
Ruspini, S.F.
Puente, V.R.
Diez, B.A.
Teijo, M.J.
Cerbino, G.N.
Varela, L.S.
Guolo, M.N.
Del Carmen Batlle, A.M.
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).
format JOUR
author Rossetti, M.V.
Buzaleh, A.M.
Parera, V.E.
Fukuda, H.
Lombardo, M.E.
Lavandera, J.
Gerez, E.N.
Melito, V.A.
Zuccoli, J.R.
Ruspini, S.F.
Puente, V.R.
Diez, B.A.
Teijo, M.J.
Cerbino, G.N.
Varela, L.S.
Guolo, M.N.
Del Carmen Batlle, A.M.
author_facet Rossetti, M.V.
Buzaleh, A.M.
Parera, V.E.
Fukuda, H.
Lombardo, M.E.
Lavandera, J.
Gerez, E.N.
Melito, V.A.
Zuccoli, J.R.
Ruspini, S.F.
Puente, V.R.
Diez, B.A.
Teijo, M.J.
Cerbino, G.N.
Varela, L.S.
Guolo, M.N.
Del Carmen Batlle, A.M.
author_sort Rossetti, M.V.
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
url http://hdl.handle.net/20.500.12110/paper_03252957_v50_n4_p547_Rossetti
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