Samenvatting
Coenzyme A is an essential metabolite known for its central role in over one hundred cellular metabolic reactions. In cells, Coenzyme A is synthesized de novo in five enzymatic steps with vitamin B5 as the starting metabolite, phosphorylated by pantothenate kinase. Mutations in the pantothenate kinase 2 gene cause a severe form of neurodegeneration for which no treatment is available. One therapeutic strategy is to generate Coenzyme A precursors downstream of the defective step in the pathway. Here we describe the synthesis, characteristics and in vivo rescue potential of the acetyl-Coenzyme A precursor S-acetyl-4'-phosphopantetheine as a possible treatment for neurodegeneration associated with pantothenate kinase deficiency.
Originele taal-2 | English |
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Artikelnummer | 11260 |
Aantal pagina's | 12 |
Tijdschrift | Scientific Reports |
Volume | 7 |
Nummer van het tijdschrift | 1 |
DOI's | |
Status | Published - 12-sep.-2017 |