Mutations Which Impede Loop/Sheet Polymerization Enhance the Secretion of Human α1-Antitrypsin Deficiency Variants
Author:
Publisher
Elsevier BV
Subject
Cell Biology,Molecular Biology,Biochemistry
Reference31 articles.
1. Amino acid substitution Glu→Lys in α1-antitrypsin PiZ
2. Purification and Partial Characterization of PAS-Positive Inclusion Bodies from the Liver in Alpha1-Antitrypsin Deficiency
3. Siiyama (serine 53 (TCC) to phenylalanine 53 (TTC)). A new alpha 1-antitrypsin-deficient variant with mutation on a predicted conserved residue of the serpin backbone
4. The mechanism of Z α1-antitrypsin accumulation in the liver
5. Effect of the Z mutation on the physical and inhibitory properties of .alpha.1-antitrypsin
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1. Alpha-1-Antitrypsin Deficiency: A Misfolded Secretory Glycoprotein Damages the Liver by Proteotoxicity and Its Reduced Secretion Predisposes to Emphysematous Lung Disease Because of Protease-Inhibitor Imbalance;Encyclopedia of Cell Biology;2023
2. Alpha 1-Antitrypsin Deficiency: A Disorder of Proteostasis-Mediated Protein Folding and Trafficking Pathways;International Journal of Molecular Sciences;2020-02-21
3. α1‐Antitrypsin Deficiency;The Liver;2020-01-24
4. Role of Oxidative Stress Induced by Cigarette Smoke in the Pathogenicity of Chronic Obstructive Pulmonary Disease;Oxidative Stress in Lung Diseases;2019-11-24
5. Functional characterization of a SNP (F51S) found in human alpha 1‐antitrypsin;Molecular Genetics & Genomic Medicine;2019-06-28
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