BAC-trap studies of Purkinje cells in normal and FMR1 mutant mice

ABSTRACT: The molecular mechanism(s) leading to Purkinje neuron loss in the neurodegenerative disorder Fragile X-Associated Tremor/Ataxia Syndrome (FXTAS) are limited by the complex morphology of this cell type. Purkinje neurons are notoriously difficult to isolate and maintain in culture presenting considerable difficultly to identify molecular changes in response to riboCGG repeat-containing mRNA that induces neurotoxicity in FXTAS. Several studies have uncovered a number of RNA binding proteins involved in translation that aberrantly interact with the toxic RNA; however, whether these interactions alter the translational profile of cells has not been investigated. Here we employ bacTRAP translational profiling to demonstrate that Purkinje neurons ectopically expressing 90 CGG repeats exhibit a dr

ORGANISM(S): Mus musculus

SUBMITTER: Chad Shaw 

PROVIDER: E-GEOD-57034 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress