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Hepatic and Extrahepatic Sources and Manifestations in Endoplasmic Reticulum Storage Diseases

Alpha-1-antitrypsin (AAT) and fibrinogen are secretory acute phase reactant proteins. Circulating AAT and fibrinogen are synthesized exclusively in the liver. Mutations in the encoding genes result in conformational abnormalities of the two molecules that aggregate within the rough endoplasmic retic...

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Detalles Bibliográficos
Autores principales:	Callea, Francesco, Francalanci, Paola, Giovannoni, Isabella
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	MDPI 2021
Materias:	Review
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8198767/ https://www.ncbi.nlm.nih.gov/pubmed/34071368 http://dx.doi.org/10.3390/ijms22115778

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