'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...
Ausführliche Beschreibung
Bibliographische Detailangaben
| Hauptverfasser: |
Mukherji, M,
Kershaw, N,
MacKinnon, C,
Clifton, I,
Wierzbicki, A,
Schofield, C,
Lloyd, MD |
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| Format: | Journal article
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| Sprache: | English |
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| Veröffentlicht: |
2001
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