Inclusion bodies and autophagosomes: are ER-derived protective organelles different than classical autophagosomes?
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.
Granell
Susana
S
Department of Biochemistry and Molecular Biology, University of Arkansas for Medical Sciences, Little Rock, Arkansas 7220, USA.
Baldini
Giulia
G
D000818:Animals; D001343:Autophagy / Q000502:physiology*; D004721:Endoplasmic Reticulum / Q000502:physiology*; D006801:Humans; D002479:Inclusion Bodies / Q000502:physiology*; D010588:Phagosomes / Q000502:physiology*; D000515:alpha 1-Antitrypsin / Q000378:metabolism
D000515:alpha 1-Antitrypsin