Article
A ketogenic diet rescues the murine succinic semialdehyde dehydrogenase deficient phenotype.
Experimental neurology - 1 Apr 2008
Nylen Kirk, Velazquez Jose Luis Perez, Likhodii Sergei S, Cortez Miguel A, Shen Lily, Leshchenko Yevgen, Adeli Khosrow, Gibson K Michael, Burnham W M, Snead O Carter
Abstract excerpt
Succinic semialdehyde dehydrogenase (SSADH) deficiency is a heritable disorder of GABA degradation characterized by ataxia, psychomotor retardation and seizures. To date, there is no effective treatment for SSADH deficiency. We tested the hypothesis that a ketogenic diet (KD) would improve outcome in an animal model of SSADH deficiency, the SSADH knockout mouse (Aldh5a1-/-). Using a 4:1 ratio of fat to combined...
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