Endophilin-A Deficiency Induces the Foxo3a-Fbxo32 Network in the Brain and Causes Dysregulation of Autophagy and the Ubiquitin-Proteasome System.
Endophilin-A, a well-characterized endocytic adaptor essential for synaptic vesicle recycling, has recently been linked to neurodegeneration. We report here that endophilin-A deficiency results in impaired movement, age-dependent ataxia, and neurodegeneration in mice. Transcriptional analysis of endophilin-A mutant mice, complemented by proteomics, highlighted ataxia- and protein-homeostasis-related genes and revealed upregulation of ... the E3-ubiquitin ligase FBXO32/atrogin-1 and its transcription factor FOXO3A. FBXO32 overexpression triggers apoptosis in cultured cells and neurons but, remarkably, coexpression of endophilin-A rescues it. FBXO32 interacts with all three endophilin-A proteins. Similarly to endophilin-A, FBXO32 tubulates membranes and localizes on clathrin-coated structures. Additionally, FBXO32 and endophilin-A are necessary for autophagosome formation, and both colocalize transiently with autophagosomes. Our results point to a role for endophilin-A proteins in autophagy and protein degradation, processes that are impaired in their absence, potentially contributing to neurodegeneration and ataxia.
Mesh Terms:
Acyltransferases, Aging, Animals, Apoptosis, Ataxia, Autophagosomes, Autophagy, Brain, Forkhead Box Protein O3, HeLa Cells, Hippocampus, Homeostasis, Humans, Male, Mice, Mice, Knockout, Movement Disorders, Muscle Proteins, Mutation, Nerve Degeneration, Parkinson Disease, Proteasome Endopeptidase Complex, Protein Binding, SKP Cullin F-Box Protein Ligases, Transcription, Genetic, Ubiquitin, Up-Regulation
Acyltransferases, Aging, Animals, Apoptosis, Ataxia, Autophagosomes, Autophagy, Brain, Forkhead Box Protein O3, HeLa Cells, Hippocampus, Homeostasis, Humans, Male, Mice, Mice, Knockout, Movement Disorders, Muscle Proteins, Mutation, Nerve Degeneration, Parkinson Disease, Proteasome Endopeptidase Complex, Protein Binding, SKP Cullin F-Box Protein Ligases, Transcription, Genetic, Ubiquitin, Up-Regulation
Cell Rep
Date: Dec. 18, 2015
PubMed ID: 27720640
View in: Pubmed Google Scholar
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