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Although there is a broad clinical spectrum of glutathione synthetase deficiency, manifestations usually include hemolytic anemia, often include metabolic acidosis, and sometimes include progressive neurologic symptoms as well as other systemic signs (eg, respiratory distress, sepsis, hyperbilirubinemia) (62

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the immunological barrier is composed of gut-associated lymphoid tissue, the physical barrier consists of epithelial TJ/AJ proteins and microbiota, and the chemical barrier is composed of antimicrobial proteins, IgA, and a mucus layer [143]

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Ubiquinone (CoQ10) mediates this inhibition