Back to Search
Start Over
Acetyl-4'-phosphopantetheine is stable in serum and prevents phenotypes induced by pantothenate kinase deficiency.
- Source :
-
Scientific reports [Sci Rep] 2017 Sep 12; Vol. 7 (1), pp. 11260. Date of Electronic Publication: 2017 Sep 12. - Publication Year :
- 2017
-
Abstract
- 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.
- Subjects :
- Animals
Cell Line
Disease Models, Animal
Drosophila
Humans
Mice
Pantetheine administration & dosage
Pantetheine chemical synthesis
Pantetheine isolation & purification
Pantetheine pharmacokinetics
Treatment Outcome
Heredodegenerative Disorders, Nervous System drug therapy
Pantetheine analogs & derivatives
Phosphotransferases (Alcohol Group Acceptor) deficiency
Serum chemistry
Subjects
Details
- Language :
- English
- ISSN :
- 2045-2322
- Volume :
- 7
- Issue :
- 1
- Database :
- MEDLINE
- Journal :
- Scientific reports
- Publication Type :
- Academic Journal
- Accession number :
- 28900161
- Full Text :
- https://doi.org/10.1038/s41598-017-11564-8