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Coenzyme Q10 modulates the immunity by enhancing mononuclear macrophage, NK cell activity, and regulating gut microbiota

J Clin Nutr 7(3):8186

Although biochemically separated from the GSH system, the Trx system shares NADPH as a common reducing cofactor and serves as an alternative pathway for reducing disulfide bonds, making this system especially relevant if the GSH system has been compromised or rendered dysfunctional (Silva-Adaya et al., 2014)

For GSD-1b patients poorly respond to regular treatment, somatic gene therapy is becoming an available choice

The primary function of the urea cycle is to process this excess ammonia into urea which can then be eliminated through excretion by the kidney