Cargando…
Molecular Mechanism of Z α1-Antitrypsin Deficiency
The Z mutation (E342K) of α1-antitrypsin (α1-AT), carried by 4% of Northern Europeans, predisposes to early onset of emphysema due to decreased functional α1-AT in the lung and to liver cirrhosis due to accumulation of polymers in hepatocytes. However, it remains unclear why the Z mutation causes in...
Autores principales: | Huang, Xin, Zheng, Ying, Zhang, Fei, Wei, Zhenquan, Wang, Yugang, Carrell, Robin W., Read, Randy J., Chen, Guo-Qiang, Zhou, Aiwu |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
American Society for Biochemistry and Molecular Biology
2016
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4957051/ https://www.ncbi.nlm.nih.gov/pubmed/27246852 http://dx.doi.org/10.1074/jbc.M116.727826 |
Ejemplares similares
-
Allosteric Modulation of Hormone Release from Thyroxine and Corticosteroid-binding Globulins
por: Qi, Xiaoqiang, et al.
Publicado: (2011) -
Structural basis for the specificity of renin-mediated angiotensinogen cleavage
por: Carrell, Robin W., et al.
Publicado: (2019) -
Calreticulin enhances the secretory trafficking of a misfolded α-1-antitrypsin
por: Mohan, Harihar Milaganur, et al.
Publicado: (2021) -
Regulation of Bone Morphogenetic Protein 9 (BMP9) by Redox-dependent Proteolysis
por: Wei, Zhenquan, et al.
Publicado: (2014) -
Structure of UBE2Z Enzyme Provides Functional Insight into Specificity in the FAT10 Protein Conjugation Machinery
por: Schelpe, Julien, et al.
Publicado: (2016)