Tetrahydrobiopterin synthesis and metabolism is impaired in dystrophin‐deficient mdx mice and humans

A. Lindsay,Bailey Kemp,Alexie A. Larson,C. Baumann,Preston M. McCourt,J. Holm,P. Karachunski,D. Lowe,J. Ervasti

Published 2021 in Acta Physiologica

ABSTRACT

Loss of dystrophin causes oxidative stress and affects nitric oxide synthase‐mediated vascular function in striated muscle. Because tetrahydrobiopterin is an antioxidant and co‐factor for nitric oxide synthase, we tested the hypothesis that tetrahydrobiopterin would be low in mdx mice and humans deficient for dystrophin.

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