Inclusion bodies and autophagosomes: are ER-derived protective organelles different than classical autophagosomes?

Autophagy
Susana Granell, Giulia Baldini

Abstract

A hallmark of some endoplasmic reticulum (ER)-storage diseases is the formation of inclusion bodies (IBs) that are membrane-limited. The nature and function of the IBs has started to be investigated. We have recently found that sequestration of mutated alpha1-antitrypsin (ATZ) into IBs is a cell protective mechanism that maintains ER function. We also found that IBs are ER-derived and yet separate from the main ER and do not have markers of autophagosomes and lysosomes. We propose that formation of the IBs is a quality control mechanism that leads to storage of unwanted proteins outside the secretory pathway by a mechanism different than direct autophagosome formation from the ER.

Citations

Jan 27, 2010·The Journal of Cell Biology·Riccardo BernasconiMaurizio Molinari
Jul 5, 2013·PloS One·Patrícia Isabel MarquesSusana Seixas
Mar 21, 2009·Annual Review of Biochemistry·Evan T PowersWilliam E Balch
Jun 1, 2010·Experimental Biology and Medicine·Shalesh KaushalJeffrey H Teckman

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