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Metabolism. 2018 Apr;81:97-112. doi: 10.1016/j.metabol.2017.11.010. Epub 2017 Nov 21.

The emerging role of immune dysfunction in mitochondrial diseases as a paradigm for understanding immunometabolism.

Author information

1
Metabolism, Infection and Immunity Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, USA.
2
Department of Pediatrics, The University of Texas Health Science Center, Houston, TX, USA.
3
Metabolism, Infection and Immunity Section, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, USA. Electronic address: peter.mcguire@nih.gov.

Abstract

Immunometabolism aims to define the role of intermediary metabolism in immune cell function, with bioenergetics and the mitochondria recently taking center stage. To date, the medical literature on mitochondria and immune function extols the virtues of mouse models in exploring this biologic intersection. While the laboratory mouse has become a standard for studying mammalian biology, this model comprises part of a comprehensive approach. Humans, with their broad array of inherited phenotypes, serve as a starting point for studying immunometabolism; specifically, patients with mitochondrial disease. Using this top-down approach, the mouse as a model organism facilitates further exploration of the consequences of mutations involved in mitochondrial maintenance and function. In this review, we will discuss the emerging phenotype of immune dysfunction in mitochondrial disease as a model for understanding the role of the mitochondria in immune function in available mouse models.

KEYWORDS:

Bioenergetics; Immunity; Mitochondria; Mouse models; Oxidative phosphorylation

PMID:
29162500
PMCID:
PMC5866745
DOI:
10.1016/j.metabol.2017.11.010
[Indexed for MEDLINE]
Free PMC Article

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