Porphyrinogenesis in rat cerebellum. Effect of high δ-aminolevulinic acid concentration

1. δ-aminolevulinic acid (ALA) uptake as well as precursor accumulation and porphyrin biosynthesis were investigated in rat cerebellum, using as experimental approach minimal tissue units called particles. 2. ALA was shown to be taken up into cerebellum particles by a non saturable process up to 4.0...

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Autores principales:	Princ, Fernando Gabriel, Juknat, Adela Ana, Batlle, Alcira María del Carmen
Publicado:	1994
Materias:	porphyria Porphyrinogenesis rat cerebellum δ-aminolevulinic acid aminolevulinic acid glucose porphobilinogen porphobilinogen deaminase porphyrin animal tissue article cerebellum controlled study drug accumulation glucose transport male nonhuman porphyrin metabolism priority journal rat Aminolevulinic Acid Animal Cerebellum Glucose Male Porphyrins Rats Support, Non-U.S. Gov't
Acceso en línea:	https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_03063623_v25_n4_p761_Princ http://hdl.handle.net/20.500.12110/paper_03063623_v25_n4_p761_Princ
Aporte de:	Biblioteca Digital - Facultad de Ciencias Exactas y Naturales (UBA) de Universidad de Buenos Aires

id	paper:paper_03063623_v25_n4_p761_Princ
record_format	dspace
spelling	paper:paper_03063623_v25_n4_p761_Princ2023-06-08T15:31:06Z Porphyrinogenesis in rat cerebellum. Effect of high δ-aminolevulinic acid concentration Princ, Fernando Gabriel Juknat, Adela Ana Batlle, Alcira María del Carmen porphyria Porphyrinogenesis rat cerebellum δ-aminolevulinic acid aminolevulinic acid glucose porphobilinogen porphobilinogen deaminase porphyrin animal tissue article cerebellum controlled study drug accumulation glucose transport male nonhuman porphyria porphyrin metabolism priority journal rat Aminolevulinic Acid Animal Cerebellum Glucose Male Porphyrins Rats Support, Non-U.S. Gov't 1. δ-aminolevulinic acid (ALA) uptake as well as precursor accumulation and porphyrin biosynthesis were investigated in rat cerebellum, using as experimental approach minimal tissue units called particles. 2. ALA was shown to be taken up into cerebellum particles by a non saturable process up to 4.0 mM ALA whereas PBG and porphyrin formation exhibited a hyperbolic response reaching the plateau at about 1.0 and 1.5 mM ALA respectively. 3. Exposure of cerebellum particles to high exogenous ALA amounts (0.01-4.0 mM) indicated that ALA can be accumulated in relatively high concentrations in the cells (40 nmol/mg protein). Under these experimental conditions, PBG-D presented a low activity (3.25 pmol/mg protein/4 hr) showing to be a secondary control step in heme biosynthesis. 4. Incubation of cerebellum particles in the presence of a physiological concentration of glucose revealed that 1.0 mM ALA decreased glucose uptake by the cells (87% during 1 hr incubation), being consistent with the fact that acute attacks are precipitated by fasting and that sugar administration appeared to be an efficient treatment of AIP crisis. 5. These findings provide the basis for a useful model to study the nature of the metabolic mechanism underlying the acute attack. © 1994. Fil:Princ, F.G. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales; Argentina. Fil:Juknat, A.A. 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. 1994 https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_03063623_v25_n4_p761_Princ http://hdl.handle.net/20.500.12110/paper_03063623_v25_n4_p761_Princ
institution	Universidad de Buenos Aires
institution_str	I-28
repository_str	R-134
collection	Biblioteca Digital - Facultad de Ciencias Exactas y Naturales (UBA)
topic	porphyria Porphyrinogenesis rat cerebellum δ-aminolevulinic acid aminolevulinic acid glucose porphobilinogen porphobilinogen deaminase porphyrin animal tissue article cerebellum controlled study drug accumulation glucose transport male nonhuman porphyria porphyrin metabolism priority journal rat Aminolevulinic Acid Animal Cerebellum Glucose Male Porphyrins Rats Support, Non-U.S. Gov't
spellingShingle	porphyria Porphyrinogenesis rat cerebellum δ-aminolevulinic acid aminolevulinic acid glucose porphobilinogen porphobilinogen deaminase porphyrin animal tissue article cerebellum controlled study drug accumulation glucose transport male nonhuman porphyria porphyrin metabolism priority journal rat Aminolevulinic Acid Animal Cerebellum Glucose Male Porphyrins Rats Support, Non-U.S. Gov't Princ, Fernando Gabriel Juknat, Adela Ana Batlle, Alcira María del Carmen Porphyrinogenesis in rat cerebellum. Effect of high δ-aminolevulinic acid concentration
topic_facet	porphyria Porphyrinogenesis rat cerebellum δ-aminolevulinic acid aminolevulinic acid glucose porphobilinogen porphobilinogen deaminase porphyrin animal tissue article cerebellum controlled study drug accumulation glucose transport male nonhuman porphyria porphyrin metabolism priority journal rat Aminolevulinic Acid Animal Cerebellum Glucose Male Porphyrins Rats Support, Non-U.S. Gov't
description	1. δ-aminolevulinic acid (ALA) uptake as well as precursor accumulation and porphyrin biosynthesis were investigated in rat cerebellum, using as experimental approach minimal tissue units called particles. 2. ALA was shown to be taken up into cerebellum particles by a non saturable process up to 4.0 mM ALA whereas PBG and porphyrin formation exhibited a hyperbolic response reaching the plateau at about 1.0 and 1.5 mM ALA respectively. 3. Exposure of cerebellum particles to high exogenous ALA amounts (0.01-4.0 mM) indicated that ALA can be accumulated in relatively high concentrations in the cells (40 nmol/mg protein). Under these experimental conditions, PBG-D presented a low activity (3.25 pmol/mg protein/4 hr) showing to be a secondary control step in heme biosynthesis. 4. Incubation of cerebellum particles in the presence of a physiological concentration of glucose revealed that 1.0 mM ALA decreased glucose uptake by the cells (87% during 1 hr incubation), being consistent with the fact that acute attacks are precipitated by fasting and that sugar administration appeared to be an efficient treatment of AIP crisis. 5. These findings provide the basis for a useful model to study the nature of the metabolic mechanism underlying the acute attack. © 1994.
author	Princ, Fernando Gabriel Juknat, Adela Ana Batlle, Alcira María del Carmen
author_facet	Princ, Fernando Gabriel Juknat, Adela Ana Batlle, Alcira María del Carmen
author_sort	Princ, Fernando Gabriel
title	Porphyrinogenesis in rat cerebellum. Effect of high δ-aminolevulinic acid concentration
title_short	Porphyrinogenesis in rat cerebellum. Effect of high δ-aminolevulinic acid concentration
title_full	Porphyrinogenesis in rat cerebellum. Effect of high δ-aminolevulinic acid concentration
title_fullStr	Porphyrinogenesis in rat cerebellum. Effect of high δ-aminolevulinic acid concentration
title_full_unstemmed	Porphyrinogenesis in rat cerebellum. Effect of high δ-aminolevulinic acid concentration
title_sort	porphyrinogenesis in rat cerebellum. effect of high δ-aminolevulinic acid concentration
publishDate	1994
url	https://bibliotecadigital.exactas.uba.ar/collection/paper/document/paper_03063623_v25_n4_p761_Princ http://hdl.handle.net/20.500.12110/paper_03063623_v25_n4_p761_Princ
work_keys_str_mv	AT princfernandogabriel porphyrinogenesisinratcerebellumeffectofhighdaminolevulinicacidconcentration AT juknatadelaana porphyrinogenesisinratcerebellumeffectofhighdaminolevulinicacidconcentration AT batllealciramariadelcarmen porphyrinogenesisinratcerebellumeffectofhighdaminolevulinicacidconcentration
_version_	1768544913049255936

Porphyrinogenesis in rat cerebellum. Effect of high δ-aminolevulinic acid concentration

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