Mental retardation genes in drosophila: New approaches to understanding and treating developmental brain disorders.
Drosophila melanogaster is emerging as a valuable genetic model system for the study of mental retardation (MR). MR genes are remarkably similar between humans and fruit flies. Cognitive behavioral assays can detect reductions in learning and memory in flies with mutations in MR genes. Neuroanatomical methods, including some at single-neuron resolution, are helping to reveal the cellular bases of faulty brain development caused by MR gene mutations. Drosophila fragile X mental retardation 1 (dfmr1) is the fly counterpart of the human gene whose malfunction causes fragile X syndrome. Research on the fly gene is leading the field in molecular mechanisms of the gene product's biological function and in pharmacological rescue of brain and behavioral phenotypes. Future work holds the promise of using genetic pathway analysis and primary neuronal culture methods in Drosophila as tools for drug discovery for a wide range of MR and related disorders.
Restifo
Linda L
LL
ARL Division of Neurobiology, University of Arizona, and Department of Neurology, Arizona Health Sciences Center, Tucson Arizona 85721-0077, USA. LLR@neurobio.arizona.edu
D000818:Animals; D001927:Brain Diseases / Q000188:drug therapy* / Q000235:genetics*; D029721:Drosophila Proteins / Q000235:genetics*; D004781:Environmental Exposure; D051860:Fragile X Messenger Ribonucleoprotein 1 / Q000235:genetics*; D005838:Genotype; D006801:Humans; D008607:Intellectual Disability / Q000209:etiology / Q000235:genetics*; D008967:Molecular Biology / Q000379:methods*; D010641:Phenotype; D017354:Point Mutation / Q000235:genetics
D029721:Drosophila Proteins; D051860:Fragile X Messenger Ribonucleoprotein 1; C467328:FMR1 protein, Drosophila