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PNAS:缺乏CFTR的新生小猪神经系统功能异常

PNAS:缺乏CFTR的新生小猪神经系统功能异常

封面故事:图为一只新生仔猪三叉神经伪彩色激光共聚焦显微镜图像。

研究者研究囊性纤维化跨膜通道调节因子(CFTR)基因是否断裂。

CFTR基因引起囊性纤维化,直接影响神经系统功能。在猪囊性纤维化模型中,研究人员发现CFTR的缺失导致髓鞘异常,轴突密度减少,神经传导速度减缓——这些变化与患有囊性纤维化神经疾病的人相一致。

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CFTR-deficient pigs display peripheral nervous system defects at birth

PNAS, February 4, 2013, doi:10.1073/pnas.1222729110

Peripheral nervous system abnormalities, including neuropathy, have been reported in people with cystic fibrosis. These abnormalities have largely been attributed to secondary manifestations of the disease. We tested the hypothesis that disruption of the cystic fibrosis transmembrane conductance regulator (CFTR) gene directly influences nervous system function by studying newborn CFTR−/− pigs. We discovered CFTR expression and activity in Schwann cells, and loss of CFTR caused ultrastructural myelin sheath abnormalities similar to those in known neuropathies. Consistent with neuropathic changes, we found increased transcripts for myelin protein zero, a gene that, when mutated, can cause axonal and/or demyelinating neuropathy. In addition, axon density was reduced and conduction velocities of the trigeminal and sciatic nerves were decreased. Moreover, in vivo auditory brainstem evoked potentials revealed delayed conduction of the vestibulocochlear nerve. Our data suggest that loss of CFTR directly alters Schwann cell function and that some nervous system defects in people with cystic fibrosis are likely primary.

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