Redox signaling in inherited diseases of metabolism

Abstract

Advances in basic and clinical research present compelling evidence that redox signaling constitutes a specific mechanism that operates at the molecular, cellular, and organ levels. The efficiency of energy producing pathways and coupling of electron transfer establish the thresholds of reactive species concentrations that may activate redox signaling events for function adjustment. A great deal of specificity in redox signaling is achieved by the reactivity of the target and the biophysical properties of its vicinity. Redox modification of proteins in circulation such as the case of Cys oxidation in albumin demonstrate that systemic conditions favorable for redox signaling could elicit organ-specific effects. Results from research on inherited metabolic diseases help to shed light on the intricate mechanisms that connect metabolism and redox signaling in more common chronic disorders such as cardiovascular disease, neurodegeneration and cancer.