A lag in intracellular degradation of mutant alpha 1-antitrypsin correlates with the liver disease phenotype in homozygous PiZZ alpha 1-antitrypsin deficiency.

Liver injury in PiZZ alpha 1-antitrypsin (alpha 1-AT) deficiency probably results from toxic effects of the abnormal alpha 1-AT molecule accumulating within the ER of liver cells. However, only 12-15% of individuals with this same genotype develops liver disease. Therefore, we predicted that other g...

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Detalhes bibliográficos

Publicado no:	Proc Natl Acad Sci U S A
Principais autores:	Wu, Y, Whitman, I, Molmenti, E, Moore, K, Hippenmeyer, P, Perlmutter, D H
Formato:	Artigo
Idioma:	Inglês
Publicado em:	National Academy of Sciences 1994
Assuntos:	Research Article
Acesso em linha:	https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC44737/ https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/8090762/ https://ncbi.nlm.nih.govhttps://doi.org/10.1073/pnas.91.19.9014
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