'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...
詳細記述
書誌詳細
主要な著者: |
Mukherji, M,
Kershaw, N,
MacKinnon, C,
Clifton, I,
Wierzbicki, A,
Schofield, C,
Lloyd, MD |
フォーマット: | Journal article
|
言語: | English |
出版事項: |
2001
|