Coproporfiria hereditaria. Estudios bioquímicos en 7 pacientes sintomáticos y familiares / Coproporphyria heredity. Studies biochemistry in 7 patients sintomatics and familiaries

Se han estudiado 7 pacientes con coproporfiria herediatria (CPH) sintomática: en 6 de ellos se investigaron 14 familiares consanguíneos. Tres de los siete (43 por ciento) pacientes exhibián signos cutáneos hiperpigmentación, fotosensibilidad, y/o máculas y todos presentaron ataques neuroviscerales. Desde el punto de vista bioquímico los pacientes excretaban niveles elevados de precursores y/o porfirinas en orina ácido 5-aminoleválico (ALA) (3,9 - 14,1 mg/24h), porfobilinógeno (PBG) (6,5 - 47,9 mg/24h), porfirinas totales (645-31129 mug/24h), que disminuyeron alcanzada la remisión (ALA: 0,2 - 5,3 mg/24h: PBG: 0,8 -25,2 mg/25,2 mg/24h, porfirinas totales 112-952 mu/24h, valor normal: ALA 2-4 mg/24h: PBG: 1-2 mg/24h. Porfirinas 20-250 mug/24h). Las profirinas fecales también estuvieron incrementadas la fase aguda (316-50.056 mug/g seco) y en remisión (165-1600) mug/g seco) valor normal <= 130 mu/g seco. El patrón de excreción de porfirinas urinarias y fecales mostró siempre una elevada concentración de coproporfirinas, la longitud de onda de emisión (618 nm) de las porfirinas plasmáticas permitió identificar la porfiria como una CPH y distinguirla de la porfiria variegata (PV). El tratamiento del ataque agudo de la CPH fue semejante al empleado en los casos de porfiria aguda intermitente o PV carbohidratos (ataques agudos 300- 500 g/diarios, ataques leves 20 - 40 g/diarios) complejo vitamínico B y ácido fólico (30 mg/día). Cuando los pacientes presentaron signos clínicos cutáneos se administró S-adenosil-L-metionina 12 mg/kg/día durante 3 semanas.

Coproporfiria hereditaria. Estudios bioquímicos en 7 pacientes sintomáticos y familiares / Coproporphyria heredity. Studies biochemistry in 7 patients sintomatics and familiaries

Se han estudiado 7 pacientes con coproporfiria herediatria (CPH) sintomática: en 6 de ellos se investigaron 14 familiares consanguíneos. Tres de los siete (43 por ciento) pacientes exhibián signos cutáneos hiperpigmentación, fotosensibilidad, y/o máculas y todos presentaron ataques neuroviscerales. Desde el punto de vista bioquímico los pacientes excretaban niveles elevados de precursores y/o porfirinas en orina ácido 5-aminoleválico (ALA) (3,9 - 14,1 mg/24h), porfobilinógeno (PBG) (6,5 - 47,9 mg/24h), porfirinas totales (645-31129 mug/24h), que disminuyeron alcanzada la remisión (ALA: 0,2 - 5,3 mg/24h: PBG: 0,8 -25,2 mg/25,2 mg/24h, porfirinas totales 112-952 mu/24h, valor normal: ALA 2-4 mg/24h: PBG: 1-2 mg/24h. Porfirinas 20-250 mug/24h). Las profirinas fecales también estuvieron incrementadas la fase aguda (316-50.056 mug/g seco) y en remisión (165-1600) mug/g seco) valor normal <= 130 mu/g seco. El patrón de excreción de porfirinas urinarias y fecales mostró siempre una elevada concentración de coproporfirinas, la longitud de onda de emisión (618 nm) de las porfirinas plasmáticas permitió identificar la porfiria como una CPH y distinguirla de la porfiria variegata (PV). El tratamiento del ataque agudo de la CPH fue semejante al empleado en los casos de porfiria aguda intermitente o PV carbohidratos (ataques agudos 300- 500 g/diarios, ataques leves 20 - 40 g/diarios) complejo vitamínico B y ácido fólico (30 mg/día). Cuando los pacientes presentaron signos clínicos cutáneos se administró S-adenosil-L-metionina 12 mg/kg/día durante 3 semanas.(AU)

[Bacteriochlorophyll a synthesis in Rhodopseudomonas palustris]. / Biosíntesis de bacterioclorofila a en Rhodopseudomonas palustris.

By means of electron microscope it was demonstrated that photosynthetically-grown Rhodopseudomonas palustris exhibits an intracytoplasmic membrane system (Figure 1a), which is not observed in aerobically-dark grown bacteria (Figure 1b). The content of bacteriochlorophyll alpha and the enzyme activity of succinil-CoA-synthetase, ALA-sinthetase and ALA-dehydratase in several media grown Rhodopseudomonas palustris could be measured. Aerobically-dark grown cells do not synthetize bacteriochlorophyll alpha and the activity of ALA-synthetase is lower than in photosynthetically-grown cells (Table 1), suggesting a possible regulatory role for this enzyme in the pigment biosynthesis. Some inhibitors of electron transport and uncouplers of photophosphorylation inhibit both the bacterial growth and bacteriochlorophyll alpha biosynthesis (Table 2), while the levels of ALA-synthetase are not affected. If the incubation in the presence of these kinds of compounds is prolongated, the effects disappear. Although the regulatory role of ALA-synthetase should be very important, apparently it would not be the unique regulatory factor for bacteriochlorophyll alpha biosynthesis in Rhodopseudomonas palustris.

Biosíntesis de bacterioclorofila a en Rhodopseudomonas palustris / [Bacteriochlorophyll a synthesis in Rhodopseudomonas palustris]

By means of electron microscope it was demonstrated that photosynthetically-grown Rhodopseudomonas palustris exhibits an intracytoplasmic membrane system (Figure 1a), which is not observed in aerobically-dark grown bacteria (Figure 1b). The content of bacteriochlorophyll alpha and the enzyme activity of succinil-CoA-synthetase, ALA-sinthetase and ALA-dehydratase in several media grown Rhodopseudomonas palustris could be measured. Aerobically-dark grown cells do not synthetize bacteriochlorophyll alpha and the activity of ALA-synthetase is lower than in photosynthetically-grown cells (Table 1), suggesting a possible regulatory role for this enzyme in the pigment biosynthesis. Some inhibitors of electron transport and uncouplers of photophosphorylation inhibit both the bacterial growth and bacteriochlorophyll alpha biosynthesis (Table 2), while the levels of ALA-synthetase are not affected. If the incubation in the presence of these kinds of compounds is prolongated, the effects disappear. Although the regulatory role of ALA-synthetase should be very important, apparently it would not be the unique regulatory factor for bacteriochlorophyll alpha biosynthesis in Rhodopseudomonas palustris.

Biosíntesis de bacterioclorofila a en Rhodopseudomonas palustris. / [Bacteriochlorophyll a synthesis in Rhodopseudomonas palustris]

By means of electron microscope it was demonstrated that photosynthetically-grown Rhodopseudomonas palustris exhibits an intracytoplasmic membrane system (Figure 1a), which is not observed in aerobically-dark grown bacteria (Figure 1b). The content of bacteriochlorophyll alpha and the enzyme activity of succinil-CoA-synthetase, ALA-sinthetase and ALA-dehydratase in several media grown Rhodopseudomonas palustris could be measured. Aerobically-dark grown cells do not synthetize bacteriochlorophyll alpha and the activity of ALA-synthetase is lower than in photosynthetically-grown cells (Table 1), suggesting a possible regulatory role for this enzyme in the pigment biosynthesis. Some inhibitors of electron transport and uncouplers of photophosphorylation inhibit both the bacterial growth and bacteriochlorophyll alpha biosynthesis (Table 2), while the levels of ALA-synthetase are not affected. If the incubation in the presence of these kinds of compounds is prolongated, the effects disappear. Although the regulatory role of ALA-synthetase should be very important, apparently it would not be the unique regulatory factor for bacteriochlorophyll alpha biosynthesis in Rhodopseudomonas palustris.