sapropterin dihydrochloride + folic acid

Sapropterin dihydrochloride (6R-BH4) is a synthetic form of the naturally occurring cofactor tetrahydrobiopterin, which is being investigated by Emory University in combination with folic acid for the treatment of chronic kidney disease (CKD). In CKD, oxidative stress and BH4 deficiency lead to the 'uncoupling' of nitric oxide synthase (NOS), resulting in decreased nitric oxide production and increased superoxide formation. This combination therapy aims to restore NOS coupling, thereby increasing nitric oxide bioavailability, improving vascular endothelial function, and reducing sympathetic nervous system overactivation. Clinical trials led by Dr. Jeanie Park have demonstrated that this treatment significantly reduces muscle sympathetic nerve activity, improves arterial stiffness, and ameliorates the exaggerated blood pressure response during exercise in patients with CKD and hypertension.

Brand names

Kuvan

Other names

tetrahydrobiopterinBH4BH-4BH 46R-BH4folatefolic acidvitamin B9