Acetyl-4′-phosphopantetheine is stable in serum and prevents phenotypes induced by pantothenate kinase deficiency

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dc.contributor.authorDi Meo, Ivanoen_ZA
dc.contributor.authorColombelli, Cristinaen_ZA
dc.contributor.authorSrinivasan, Balajien_ZA
dc.contributor.authorDe Villiers, Marianneen_ZA
dc.contributor.authorHamada, Jeffreyen_ZA
dc.contributor.authorJeong, Suh Y.en_ZA
dc.contributor.authorFox, Rachelen_ZA
dc.contributor.authorWoltjer, Randall L.en_ZA
dc.contributor.authorTepper, Pieter G.en_ZA
dc.contributor.authorLahaye, Liza L.en_ZA
dc.contributor.authorRizzetto, Emanuelaen_ZA
dc.contributor.authorHarrs, Clara H.en_ZA
dc.contributor.authorDe Boer, Theoen_ZA
dc.contributor.authorVan der Zwaag, Marianneen_ZA
dc.contributor.authorJenko, Brankoen_ZA
dc.contributor.authorCusak, Alenen_ZA
dc.contributor.authorPahor, Jercaen_ZA
dc.contributor.authorKosec, Gregoren_ZA
dc.contributor.authorGrzeschik, Nicola A.en_ZA
dc.contributor.authorHayflick, Susan J.en_ZA
dc.contributor.authorTiranti, Valeriaen_ZA
dc.contributor.authorSibon, Ody C. M.en_ZA
dc.date.accessioned2018-11-30T07:01:01Z
dc.date.available2018-11-30T07:01:01Z
dc.date.issued2017-09-12
dc.descriptionCITATION: Di Meo, I., et al. 2017. Acetyl-4′-phosphopantetheine is stable in serum and prevents phenotypes induced by pantothenate kinase deficiency. Scientific Reports, 7:11260, doi:10.1038/s41598-017-11564-8.en_ZA
dc.descriptionThe original publication is available at https://www.nature.comen_ZA
dc.description.abstractCoenzyme 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.en_ZA
dc.description.urihttps://www.nature.com/articles/s41598-017-11564-8
dc.description.versionPublisher's versionen_ZA
dc.format.extent12 pages : illustrationsen_ZA
dc.identifier.citationDi Meo, I., et al. 2017. Acetyl-4′-phosphopantetheine is stable in serum and prevents phenotypes induced by pantothenate kinase deficiency. Scientific Reports, 7:11260, doi:10.1038/s41598-017-11564-8en_ZA
dc.identifier.issn2045-2322 (online)
dc.identifier.otherdoi:10.1038/s41598-017-11564-8
dc.identifier.urihttp://hdl.handle.net/10019.1/104754
dc.language.isoen_ZAen_ZA
dc.publisherNature Researchen_ZA
dc.rights.holderAuthors retain copyrighten_ZA
dc.subjectCoenzyme Aen_ZA
dc.subjectNeurodegeneration -- Treatmenten_ZA
dc.subjectCoenzyme A --Characteristicsen_ZA
dc.subjectCellular metabolic reactionsen_ZA
dc.subjectPantothenate kinase deficiencyen_ZA
dc.titleAcetyl-4′-phosphopantetheine is stable in serum and prevents phenotypes induced by pantothenate kinase deficiencyen_ZA
dc.typeArticleen_ZA
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