Deficiency of the zinc finger protein ZFP106 causes motor and sensory neurodegeneration

摘要: Zinc finger motifs are distributed amongst many eukaryotic protein families, directing nucleic acid-protein and protein-protein interactions. 106 (ZFP106) has previously been associated with roles in immune response, muscle differentiation, testes development DNA damage, although little is known about its specific function. To further investigate the function of ZFP106, we performed an in-depth characterization Zfp106 deficient mice (Zfp106(-/-)), report a novel role for ZFP106 motor sensory neuronal maintenance survival. Zfp106(-/-) develop severe abnormalities, major deficits strength histopathological changes muscle. Intriguingly, despite being highly expressed throughout central nervous system, undergo selective axonal degeneration to spinal cord peripheral system. Neurodegeneration does not occur during mice, suggesting that likely required mature neurons. Analysis embryonic neurons revealed mitochondrial function, inhibition Complex I within electron transport chain. Our results highlight vital neuron reveal player dysfunction neurodegeneration.