Back to Search
Start Over
Loss of the Neuron-Specific F-Box Protein FBXO41 Models an Ataxia-Like Phenotype in Mice with Neuronal Migration Defects and Degeneration in the Cerebellum
- Publication Year :
- 2015
- Publisher :
- Society for Neuroscience, 2015.
-
Abstract
- The cerebellum is crucial for sensorimotor coordination. The cerebellar architecture not only requires proper development but also long-term integrity to ensure accurate functioning. Developmental defects such as impaired neuronal migration or neurodegeneration are thus detrimental to the cerebellum and can result in movement disorders including ataxias. In this study, we identify FBXO41 as a novel CNS-specific F-box protein that localizes to the centrosome and the cytoplasm of neurons and demonstrate that cytoplasmic FBXO41 promotes neuronal migration. Interestingly, deletion of the FBXO41 gene results in a severely ataxic gait in mice, which show delayed neuronal migration of granule neurons in the developing cerebellum in addition to deformities and degeneration of the mature cerebellum. We show that FBXO41 is a critical factor, not only for neuronal migration in the cerebellum, but also for its long-term integrity.
- Subjects :
- Cerebellum
Ataxia
Cell Survival
Green Fluorescent Proteins
Mice, Transgenic
Nerve Tissue Proteins
Biology
Mice
Cell Movement
medicine
Animals
Humans
Spinocerebellar Ataxias
Ataxic Gait
Cells, Cultured
Neurons
General Neuroscience
F-Box Proteins
Neurodegeneration
Brain
Gene Expression Regulation, Developmental
Articles
medicine.disease
Embryo, Mammalian
Phenotype
Disease Models, Animal
medicine.anatomical_structure
CXCL3
nervous system
Animals, Newborn
Centrosome
Mutation
Neuron
medicine.symptom
Neuroscience
Neuroglia
Subcellular Fractions
Subjects
Details
- Language :
- English
- Database :
- OpenAIRE
- Accession number :
- edsair.doi.dedup.....5a8f6d5383ef58dd603003ab485d24ec