Dietary rescue of fumble--a Drosophila model for pantothenate-kinase-associated neurodegeneration.

Abstract	Hallervorden-Spatz syndrome (HSS) is a devastating neurological disease, characterized by iron accumulation in the globus pallidus in the basal ganglia. Most HSS cases are caused by mutations in one of the four human pantothenate kinases (PANK2). This PANK2-caused subgroup of HSS is sometimes referred as PKAN (pantothenate-kinase-associated neurodegeneration). No effective treatment for PKAN or HSS is currently available. fumble, a Drosophila mutant that carries a mutation in Drosophila Pank, has many features similar to those of PKAN patients. In this study, we used fumble as a model to evaluate various compounds or nutritional products for their possible therapeutic efficacy. While no product was found to dramatically improve the symptoms, GKE (containing Ginkgo biloba extract and flavone) and vitamin E showed statistically significant beneficial effects. Our studies indicate that pantothenate is of limited value in alleviating fumble phenotypes and also suggest that some compounds might have deleterious effects.
Authors	Y Yang, Z Wu, Y M Kuo, B Zhou
Journal	Journal of inherited metabolic disease (J Inherit Metab Dis) Vol. 28 Issue 6 Pg. 1055-64 ( 2005) ISSN: 0141-8955 [Print] United States
PMID	16435199 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Chemical References	Vitamin E Inosine Adenosine Triphosphate Edetic Acid Iron Phosphotransferases (Alcohol Group Acceptor) pantothenate kinase Creatine Ascorbic Acid Carnitine Acetylcysteine
Topics	Acetylcysteine (metabolism) Adenosine Triphosphate (metabolism) Animal Feed Animals Ascorbic Acid (metabolism) Carnitine (metabolism) Cloning, Molecular Creatine (metabolism) Diet Disease Models, Animal Drosophila Drosophila melanogaster Edetic Acid (chemistry) Ginkgo biloba (metabolism) Heterozygote Humans Inosine (metabolism) Iron (metabolism) Mutation Neurodegenerative Diseases (drug therapy, genetics) Phenotype Phosphotransferases (Alcohol Group Acceptor) (genetics) Transgenes Vitamin E (metabolism)

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