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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...
Autores principales: | Callea, Francesco, Francalanci, Paola, Giovannoni, Isabella |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2021
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Materias: | |
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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