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Selected metabolic aspects of elastin and collagen fiber proteolysis in diseases of the respiratory system – the significance of α1 antitrypsin deficiency

The process of elastin and collagen fiber destruction was presented based on the example of the changes taking place in the course of chronic obstructive pulmonary disease and primary spontaneous pneumothorax. In 1963, when analyzing patients with α1 antitrypsin deficiency, Dr Laurell and Dr Eriksso...

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Detalles Bibliográficos
Autores principales: Dżeljilji, Agata, Rokicki, Wojciech, Karuś, Krzysztof
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Termedia Publishing House 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5071592/
https://www.ncbi.nlm.nih.gov/pubmed/27785139
http://dx.doi.org/10.5114/kitp.2016.62614
Descripción
Sumario:The process of elastin and collagen fiber destruction was presented based on the example of the changes taking place in the course of chronic obstructive pulmonary disease and primary spontaneous pneumothorax. In 1963, when analyzing patients with α1 antitrypsin deficiency, Dr Laurell and Dr Eriksson hypothesized that elastolysis plays a role in the pathogenesis of emphysema, which marked the beginning of studies aimed at analyzing this process. The present work concerns new scientific reports regarding the hypothesis. The most important risk factors include protease-antiprotease imbalance and α1 antitrypsin protein deficiency.