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LMAN1–MCFD2 complex is a cargo receptor for the ER-Golgi transport of α1-antitrypsin
α1-antitrypsin (AAT) is a serine protease inhibitor synthesized in hepatocytes and protects the lung from damage by neutrophil elastase. AAT gene mutations result in AAT deficiency (AATD), which leads to lung and liver diseases. The AAT Z variant forms polymer within the endoplasmic reticulum (ER) o...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Portland Press Ltd.
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9022998/ https://www.ncbi.nlm.nih.gov/pubmed/35322856 http://dx.doi.org/10.1042/BCJ20220055 |