title: 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.affiliation: Department of Biological Sciences and Biotechnology, Tsinghua University, Beijing 100084, China.
authors.forename: Y
authors.initials: Y
authors.lastname: Yang
authors.forename: Z
authors.initials: Z
authors.lastname: Wu
authors.forename: Y M
authors.initials: YM
authors.lastname: Kuo
authors.forename: B
authors.initials: B
authors.lastname: Zhou
mesh_terms: D000111:Acetylcysteine / Q000378:metabolism; D000255:Adenosine Triphosphate / Q000378:metabolism; D000821:Animal Feed; D000818:Animals; D001205:Ascorbic Acid / Q000378:metabolism; D002331:Carnitine / Q000378:metabolism; D003001:Cloning, Molecular; D003401:Creatine / Q000378:metabolism; D004032:Diet; D004195:Disease Models, Animal; D004330:Drosophila; D004331:Drosophila melanogaster; D004492:Edetic Acid / Q000737:chemistry; D020441:Ginkgo biloba / Q000378:metabolism; D006579:Heterozygote; D006801:Humans; D007288:Inosine / Q000378:metabolism; D007501:Iron / Q000378:metabolism; D009154:Mutation; D019636:Neurodegenerative Diseases / Q000188:drug therapy* / Q000235:genetics*; D010641:Phenotype; D017853:Phosphotransferases (Alcohol Group Acceptor) / Q000235:genetics*; D019076:Transgenes; D014810:Vitamin E / Q000378:metabolism
chemical_list: D014810:Vitamin E; D007288:Inosine; D000255:Adenosine Triphosphate; D004492:Edetic Acid; D007501:Iron; D017853:Phosphotransferases (Alcohol Group Acceptor); C023088:pantothenate kinase; D003401:Creatine; D001205:Ascorbic Acid; D002331:Carnitine; D000111:Acetylcysteine