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Molecular and genetic analysis of the Drosophila model of fragile X syndrome.
- Source :
-
Results and problems in cell differentiation [Results Probl Cell Differ] 2012; Vol. 54, pp. 119-56. - Publication Year :
- 2012
-
Abstract
- The Drosophila genome contains most genes known to be involved in heritable disease. The extraordinary genetic malleability of Drosophila, coupled to sophisticated imaging, electrophysiology, and behavioral paradigms, has paved the way for insightful mechanistic studies on the causes of developmental and neurological disease as well as many possible interventions. Here, we focus on one of the most advanced examples of Drosophila genetic disease modeling, the Drosophila model of Fragile X Syndrome, which for the past decade has provided key advances into the molecular, cellular, and behavioral defects underlying this devastating disorder. We discuss the multitude of RNAs and proteins that interact with the disease-causing FMR1 gene product, whose function is conserved from Drosophila to human. In turn, we consider FMR1 mechanistic relationships in non-neuronal tissues (germ cells and embryos), peripheral motor and sensory circuits, and central brain circuits involved in circadian clock activity and learning/memory.
- Subjects :
- Animals
Drosophila anatomy & histology
Drosophila Proteins genetics
Drosophila Proteins metabolism
Fragile X Mental Retardation Protein genetics
Fragile X Mental Retardation Protein metabolism
Humans
MicroRNAs genetics
MicroRNAs metabolism
Mushroom Bodies anatomy & histology
Mushroom Bodies physiology
Proteome analysis
Disease Models, Animal
Drosophila genetics
Drosophila physiology
Fragile X Syndrome physiopathology
Subjects
Details
- Language :
- English
- ISSN :
- 0080-1844
- Volume :
- 54
- Database :
- MEDLINE
- Journal :
- Results and problems in cell differentiation
- Publication Type :
- Academic Journal
- Accession number :
- 22009350
- Full Text :
- https://doi.org/10.1007/978-3-642-21649-7_7