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The hypothesis has expanded to include vascular dysfunction, oxidative stress, microglial activation, and impaired proteolysis (Roda et al., 2022)

Introduction The innate immune response constitutes the first line of defense against invading pathogens, and relies on pattern recognition receptors (PRR)s for detection of infections through recognition of either molecular structures specific for non-self, or aberrant localization of molecules used by both host and microbe [1], [2]

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Efficacy and safety of pegzilarginase in arginase 1 deficiency (PEACE): a phase 3, randomized, double-blind, placebo-controlled, multi-centre trial