Artículo

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 " (2016) Acta Bioquimica Clinica Latinoamericana. 50(4):547-573
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Abstract:

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).

Registro:

Documento: Artículo
Título:Heme metabolism: The janus nature of precursorsand porphyrins
Autor: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.
Filiación:Centro de Investigaciones Sobre Porfirinas y Porfirias (CIPYP), Consejo Nacional de Investigaciones Científicas y Técnicas (CONICET), Hospital de Clínicas José de San Martín, Universidad de Buenos Aires (UBA), Viamonte 188l 10 A, Buenos Aires, C1056ABA, Argentina
Departamento de Química Biológica, Facultad de Ciencias Exactas y Naturales, Universidad de Buenos Aires (UBA), Argentina
Palabras clave:D-aminolevulinic acid; Heme metabolism; Photodynamic therapy; Porphyrias; Porphyrins; Trypanosoma cruzi; Volatile anaesthetics
Año:2016
Volumen:50
Número:4
Página de inicio:547
Página de fin:573
Título revista:Acta Bioquimica Clinica Latinoamericana
Título revista abreviado:Acta Bioquim. Clin. Latinoam.
ISSN:03252957
CODEN:ABCLD
Registro:https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_03252957_v50_n4_p547_Rossetti

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Citas:

---------- APA ----------
Rossetti, M.V., Buzaleh, A.M., Parera, V.E., Fukuda, H., Lombardo, M.E., Lavandera, J., Gerez, E.N.,..., Del Carmen Batlle, A.M. (2016) . Heme metabolism: The janus nature of precursorsand porphyrins . Acta Bioquimica Clinica Latinoamericana, 50(4), 547-573.
Recuperado de https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_03252957_v50_n4_p547_Rossetti [ ]
---------- CHICAGO ----------
Rossetti, M.V., Buzaleh, A.M., Parera, V.E., Fukuda, H., Lombardo, M.E., Lavandera, J., et al. "Heme metabolism: The janus nature of precursorsand porphyrins " . Acta Bioquimica Clinica Latinoamericana 50, no. 4 (2016) : 547-573.
Recuperado de https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_03252957_v50_n4_p547_Rossetti [ ]
---------- MLA ----------
Rossetti, M.V., Buzaleh, A.M., Parera, V.E., Fukuda, H., Lombardo, M.E., Lavandera, J., et al. "Heme metabolism: The janus nature of precursorsand porphyrins " . Acta Bioquimica Clinica Latinoamericana, vol. 50, no. 4, 2016, pp. 547-573.
Recuperado de https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_03252957_v50_n4_p547_Rossetti [ ]
---------- VANCOUVER ----------
Rossetti, M.V., Buzaleh, A.M., Parera, V.E., Fukuda, H., Lombardo, M.E., Lavandera, J., et al. Heme metabolism: The janus nature of precursorsand porphyrins . Acta Bioquim. Clin. Latinoam. 2016;50(4):547-573.
Available from: https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_03252957_v50_n4_p547_Rossetti [ ]