Acute intermittent porphyria, givosiran, and homocysteine

Acute intermittent porphyria (AIP) is a rare genetic metabolic disease caused by a specific enzyme dysfunction in the hepatic heme biosynthesis pathway. Besides the obvious clinical relevance of a disease in which patients are at risk of acute life-threatening neurovisceral attacks, the study of thi...

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Detalles Bibliográficos
Autores: Fontanellas-Romá, A. (Antonio)|||/items/4ca7871d-480e-4841-b383-6a0da85f3a81, Avila, M.A. (Matías Antonio)|||/items/3ad9abbb-c18d-445b-86cf-cb76be15419f, Arranz, E. (Elena)|||/items/116450ff-b1fa-4611-b401-4c96930faedd, Enriquez-de-Salamanca, R. (Rafael)|||/items/c206c76f-ea31-42b2-affe-d3d47b8baa6f, Morales, M. (Montserrat)|||/items/d545226f-a877-4ac4-8b94-ce1cb07f9e4b
Tipo de recurso: artículo
Fecha de publicación:2021
País:España
Institución:Universidad de Navarra
Repositorio:Dadun. Depósito Académico Digital de la Universidad de Navarra
Idioma:inglés
OAI Identifier:oai:dadun.unav.edu:10171/114733
Acceso en línea:https://hdl.handle.net/10171/114733
Access Level:acceso abierto
Palabra clave:Acute intermittent porphyria
Givosiran
Homocysteine
Descripción
Sumario:Acute intermittent porphyria (AIP) is a rare genetic metabolic disease caused by a specific enzyme dysfunction in the hepatic heme biosynthesis pathway. Besides the obvious clinical relevance of a disease in which patients are at risk of acute life-threatening neurovisceral attacks, the study of this disorder is of particular interest because it can provide straightforward proof-of-concept for new molecular therapies and also reveal unexpected metabolic crosstalks.