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Mysteries of α1-antitrypsin deficiency: emerging therapeutic strategies for a challenging disease
The classical form of α1-antitrypsin deficiency (ATD) is an autosomal co-dominant disorder that affects ~1 in 3000 live births and is an important genetic cause of lung and liver disease. The protein affected, α1-antitrypsin (AT), is predominantly derived from the liver and has the function of inhib...
Autores principales: | Ghouse, Raafe, Chu, Andrew, Wang, Yan, Perlmutter, David H. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
The Company of Biologists Limited
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3974452/ https://www.ncbi.nlm.nih.gov/pubmed/24719116 http://dx.doi.org/10.1242/dmm.014092 |
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