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Cells 2017, 6(3), 16; doi:10.3390/cells6030016

Assessing Autophagy in Mouse Models and Patients with Systemic Autoimmune Diseases

1
Centre National de la Recherche Scientifique (CNRS), Immunopathology and Therapeutic Chemistry/Laboratory of Excellence Medalis, Institut de Biologie Moléculaire et Cellulaire, Strasbourg 67000, France
2
University of Strasbourg Institute for Advanced Study (USIAS), Strasbourg 67000, France
*
Author to whom correspondence should be addressed.
Received: 27 May 2017 / Revised: 26 June 2017 / Accepted: 26 June 2017 / Published: 28 June 2017
(This article belongs to the Special Issue Assays to Monitor Autophagy in Model Systems)
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Abstract

Autophagy is a tightly regulated mechanism that allows cells to renew themselves through the lysosomal degradation of proteins, which are misfolded or produced in excess, and of damaged organelles. In the context of immunity, recent research has specially attempted to clarify its roles in infection, inflammation and autoimmunity. Autophagy has emerged as a spotlight in several molecular pathways and trafficking events that participate to innate and adaptive immunity. Deregulation of autophagy has been associated to several autoimmune diseases, in particular to systemic lupus erythematosus. Nowadays, however, experimental data on the implication of autophagy in animal models of autoimmunity or patients remain limited. In our investigations, we use Murphy Roths Large (MRL)/lymphoproliferation (lpr) lupus-prone mice as a mouse model for lupus and secondary Sjögren’s syndrome, and, herein, we describe methods applied routinely to analyze different autophagic pathways in different lymphoid organs and tissues (spleen, lymph nodes, salivary glands). We also depict some techniques used to analyze autophagy in lupus patient’s blood samples. These methods can be adapted to the analysis of autophagy in other mouse models of autoinflammatory diseases. The understanding of autophagy implication in autoimmune diseases could prove to be very useful for developing novel immunomodulatory strategies. Our attention should be focused on the fact that autophagy processes are interconnected and that distinct pathways can be independently hyper-activated or downregulated in distinct organs and tissues of the same individual. View Full-Text
Keywords: macroautophagy; chaperone-mediated autophagy; systemic lupus erythematous; Sjögren’s syndrome; autoimmunity; MRL/lpr mice; autophagy markers; salivary glands macroautophagy; chaperone-mediated autophagy; systemic lupus erythematous; Sjögren’s syndrome; autoimmunity; MRL/lpr mice; autophagy markers; salivary glands
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. (CC BY 4.0).

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Wang, F.; Li, B.; Schall, N.; Wilhelm, M.; Muller, S. Assessing Autophagy in Mouse Models and Patients with Systemic Autoimmune Diseases. Cells 2017, 6, 16.

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