Hypoxia ameliorates brain hyperoxia and NAD⁺ deficiency in a murine model of Leigh syndrome.

ABSTRACT: Leigh syndrome is a severe mitochondrial neurodegenerative disease with no effective treatment. In the Ndufs4^-/- mouse model of Leigh syndrome, continuously breathing 11% O₂ (hypoxia) prevents neurodegeneration and leads to a dramatic extension (~5-fold) in lifespan. We investigated the effect of hypoxia on the brain metabolism of Ndufs4^-/- mice by studying blood gas tensions and metabolite levels in simultaneously sampled arterial and cerebral internal jugular venous (IJV) blood. Relatively healthy Ndufs4^-/- and wildtype (WT) mice breathing air until postnatal age ~38 d were compared to Ndufs4^-/- and WT mice breathing air until ~38 days old followed by 4-weeks of breathing 11% O₂. Compared to WT control mice, Ndufs4^-/-

SUBMITTER: Grange RMH 

PROVIDER: S-EPMC8489256 | biostudies-literature | 2021 May

REPOSITORIES: biostudies-literature