'Chemical co-substrate rescue' of phytanoyl-CoA 2-hydroxylase mutants causing Refsum's Disease
The in vitro catalytic activity of two clinically observed mutants of phytanoyl-CoA 2-hydroxylase, an iron(II)/2-oxoglutarate-dependent oxygenase causing Refsum's Disease, was partially rescued by the use of alternatives to the natural cosubstrate, 2-oxoglutarate; this is the first demonstratio...
Principais autores: | Mukherji, M, Kershaw, N, MacKinnon, C, Clifton, I, Wierzbicki, A, Schofield, C, Lloyd, MD |
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Formato: | Journal article |
Idioma: | English |
Publicado em: |
2001
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