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Abstract
Tetrahydrobiopterin (BH4) is an endogenous cofactor for various enzymatic conversions of essential biomolecules including nitric oxide, tyrosine, dopamine, serotonin and phenylalanine. Depending on the physiological functions of these molecules, BH4 plays multiple roles in the cardiovascular, immune, nervous and endocrine systems. A deficiency of BH4 or an imbalance of the redox state of biopterin has been implicated in various cardiovascular and metabolic diseases. Therefore, supplementation with BH4 is considered as a therapeutic option for these diseases. In addition to the classical nitric oxide synthase (NOS)-dependent role of BH4, recent studies proposed novel NOS-independent roles of BH4 in health and disease conditions. This article reviews the updated role of BH4 in mitochondrial regulation, energy metabolism and cardiovascular and metabolic diseases.
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