Article
The endoplasmic reticulum remains functionally connected by vesicular transport after its fragmentation in cells expressing Z-α1-antitrypsin.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 Dec 2016
Dickens Jennifer A, Ordóñez Adriana, Chambers Joseph E, Beckett Alison J, Patel Vruti, Malzer Elke, Dominicus Caia S, Bradley Jayson, Peden Andrew A, Prior Ian A, Lomas David A, Marciniak Stefan J
Abstract excerpt
α1-Antitrypsin is a serine protease inhibitor produced in the liver that is responsible for the regulation of pulmonary inflammation. The commonest pathogenic gene mutation yields Z-α1-antitrypsin, which has a propensity to self-associate forming polymers that become trapped in inclusions of endoplasmic reticulum (ER). It is unclear whether these inclusions are connected to the main ER network in...
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