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Updated: Mar 28, 2026

Measurement of Heme Synthesis Levels in Mammalian Cells
Published on: July 9, 2015
[Tetrahydrobiopterin Synthesis and Biological Function]
Abstract:
Tetrahydrobiopterin (BH4) is an essential cofactor for aromatic amino acid hydroxylases and all three nitric oxide synthase (NOS) isoforms. It has protective effects as an antioxidant and scavenger of reactive nitrogen and oxygen species. It also serves as a cofactor in both normal physiologic and pathological states. In conditions of BH4 deficiency, endothelial nitric oxide synthase (eNOS) becomes 'uncoupled', which leads to endothelial dysfunction in diabetes, pulmonary hypertension and pathologic cardiac remodeling. In this review, we will discuss the pathophysiological role of BH4 in those diseases mentioned above.
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