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El control enzimático de la ingesta de fenilalanina en la fenilcetonuria
Lancet (London, England)
|February 23, 1980
Resumen
La enzima vegetal fenilalanina amoníaco liasa (PAL) reduce efectivamente los niveles de fenilalanina en la sangre en adultos sanos y pacientes con fenilcetonúricos (PKU). Esta enzima se muestra prometedora como un tratamiento potencial para la PKU mediante el manejo de la fenilalanina dietética.
Área de la Ciencia:
- La bioquímica es la bioquímica.
- Enzimología Enzimología.
- Trastornos metabólicos Los trastornos metabólicos son trastornos metabólicos que se producen en el cuerpo.
Sus antecedentes:
- La fenilalanina amoníaco liasa (PAL) es una enzima vegetal capaz de descomponer la fenilalanina.
- Los niveles elevados de fenilalanina en sangre son característicos de la fenilcetonuria (PKU), un trastorno metabólico genético.
- Las estrategias actuales de manejo de la PKU se centran en la restricción dietética de la fenilalanina.
Objetivo del estudio:
- Para investigar la eficacia de la fenilalanina amoniaca liasa (PAL) oral en la reducción de los niveles de fenilalanina en sangre postprandial.
- Para evaluar la supervivencia y la actividad de la enzima vegetal PAL dentro del tracto gastrointestinal humano.
- Evaluar la aplicación terapéutica potencial de PAL en el manejo de la fenilcetonuria (PKU).
Principales métodos:
- Administración de una preparación enzimática de fenilalanina amoníaco liasa (PAL) a adultos sanos y pacientes con fenilcetonuria (PKU).
- Monitoreo de las concentraciones de fenilalanina en sangre después de las comidas que contienen proteínas.
- Un estudio de caso que involucró a un paciente con PKU no tratado que recibió PAL tres veces al día durante 12 días consecutivos.
Principales resultados:
- La enzima vegetal PAL demostró supervivencia en el intestino, lo que lleva al agotamiento de la fenilalanina de la proteína dietética.
- Se observó una reducción significativa en el aumento postprandial de la fenilalanina en sangre tanto en adultos sanos como en pacientes con PKU.
- En un paciente con PKU no tratada, la administración diaria de PAL durante 12 días dio como resultado una reducción promedio del 25% en los niveles de fenilalanina en sangre.
Conclusiones:
- La administración oral de fenilalanina amoníaca liasa (PAL) puede reducir eficazmente los niveles de fenilalanina en sangre mediante la degradación de la fenilalanina dietética.
- La preparación enzimática muestra potencial como un nuevo enfoque terapéutico para el manejo de la fenilcetonuria (PKU).
- Se necesitan más estudios clínicos para establecer la seguridad y eficacia a largo plazo de PAL como tratamiento para la PKU.
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