Impaired Energy Production Contributes to Development of Failure in Taurine Deficient Heart

Abstract

Taurine forms a conjugate in the mitochondria with a uridine residue in the wobble position of tRNA. The resulting product, 5-taurinomethyluridine tRNA, increases the interaction between the UUG codon and AAU anticodon of tRNA, thereby improving the decoding of the UUG codon. We have shown that the protein most affected by the taurine conjugation product is ND6, which is a subunit of complex I of the respiratory chain. Thus, taurine deficiency exhibits reduced respiratory chain function. Based on these findings, we proposed that the taurine deficient heart is energy deficient. To test this idea, hearts were perfused with buffer containing acetate and glucose as substrates. The utilization of both substrates, as well as the utilization of endogenous lipids, was significantly reduced in the taurine deficient heart. This led to a 25% decrease in ATP production, an effect primarily caused by diminished aerobic metabolism and respiratory function. In addition, inefficient oxidative phosphorylation causes a further decrease in ATP generation. The data support the idea that reductions in energy metabolism, including oxidative phosphorylation, ATP generation and high energy phosphate content, contribute to the severity of the cardiomyopathy. The findings are also consistent with the hypothesis that taurine deficiency and reduced myocardial energy content increases mortality of the taurine deficient, failing heart. The clinical implications of these findings are addressed.

Metabolomic biomarkers from patients with Barth syndrome treated with elamipretide: insights from the TAZPOWER study

“…An estimated 70% of total body taurine is localized to muscles and plays vital roles in mitochondrial, muscular, and cardiovascular functions [26]. Deficiency of taurine has been linked to impaired mitochondrial energy production and decreased exercise performance and can result in cardiomyopathy [27]. In patients with Barth syndrome, plasma taurine was reported to be 36% lower than age-matched control values ( p = 0.07) [9].…”

“…Taurine is a non-proteogenic amino acid that is considered essential for bioenergetics and has been shown to be integral for heart and skeletal muscle physiology [26]. Deficiency in taurine is associated with the pathological development of a dilated cardiomyopathy [27], providing rationale for the use of taurine supplementation for patients with congestive heart failure [45]. Plasma taurine was reported to be depressed by roughly 40% in Barth syndrome compared to age-matched controls [9].…”

Effects of quercetin on cadmium-induced toxicity in rat urine using metabonomics techniques

“…[16][17][18][19][20] Taurine deficiency increases the NADH/ NAD ratio and affects energy metabolism. 21,22 PCr, a fast and high-energy phosphate molecule, is essential for intracellular energy transfer systems. 23 When the ATP in the body is lacking, PCr will be decomposed into the ATP.…”

Metabolic Reprogramming, Gut Dysbiosis, and Nutrition Intervention in Canine Heart Disease

“…Taurine has been implicated in the maintenance of normal contractile function, modulation of myocardial calcium homeostasis, and potentially acts as an antioxidant and anti-inflammatory agent ( 123 , 128 ). Schaffer et al demonstrated that the taurine-deficient heart is associated with reduced ATP generation and is energy starved, possibly due to impaired mitochondrial respiratory chain activity, and NADH utilization ( 129 ). Although taurine deficiency causes reversible cardiomyopathy in cats ( 130 ), it does not play a significant role in the development of cardiomyopathy in dogs ( 91 ).…”