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In addition, intact mitochondrial calcium homeostasis serves as an important protective barrier by confining potentially harmful mitochondrial contents, including cytochrome c, apoptosis-inducing factor (AIF), and mtDNA fragments, within the organelle and preventing their release into the cytosol, where they could trigger inflammation, necrosis, or abnormal apoptosis (see Figure 2)
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The most direct mechanism involves mutations in the lipid A biosynthesis genes lpxA , lpxC , and lpxD , which can result in partial or complete loss of LPS
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