| First Author | Fernández-Chacón R | Year | 2004 |
| Journal | Neuron | Volume | 42 |
| Issue | 2 | Pages | 237-51 |
| PubMed ID | 15091340 | Mgi Jnum | J:91702 |
| Mgi Id | MGI:3050249 | Doi | 10.1016/s0896-6273(04)00190-4 |
| Citation | Fernandez-Chacon R, et al. (2004) The synaptic vesicle protein CSP alpha prevents presynaptic degeneration. Neuron 42(2):237-51 |
| abstractText | Cysteine string protein alpha (CSPalpha)--an abundant synaptic vesicle protein that contains a DNA-J domain characteristic of Hsp40 chaperones--is thought to regulate Ca2+ channels and/or synaptic vesicle exocytosis. We now show that, in young mice, deletion of CSPalpha does not impair survival and causes no significant changes in presynaptic Ca2+ currents or synaptic vesicle exocytosis as measured in the Calyx of Held synapse. At 2-4 weeks of age, however, CSPalpha-deficient mice develop a progressive, fatal sensorimotor disorder. The neuromuscular junctions and Calyx synapses of CSPalpha-deficient mice exhibit increasing neurodegenerative changes, synaptic transmission becomes severely impaired, and the mutant mice die at approximately 2 months of age. Our data suggest that CSPalpha is not essential for the normal operation of Ca2+ channels or exocytosis but acts as a presynaptic chaperone that maintains continued synaptic function, raising the possibility that enhanced CSPalpha function could attenuate neurodegenerative diseases. |
