| 4 | 1A型夏科-马里-图思病患者施万细胞的ErbB2和ErbB3受体过度表达显示文摘Axon-derived neuregulins (NRGs) are a family of growth factors whose binding to ErbB tyrosine kinase receptors promotes the maturation, proliferation and survival of Schwann cells (SCs). Correct NRG/ ErbB signaling is essential for the homeostasis of axonal-glial complexes and seems to play a role in peripheral nerve repair. The potential involvement of ErbB receptors in human peripheral neuropathies has not been clarified. Therefore, we assessed the immunoreactivity for EGFR(ErbB1), ErbB2, and ErbB3 in nerve biopsies from patients with different for ms of Charcot-Marie-Tooth disease, type 1, (CMT1), as compared to others with inflammatory neuropathies and controls. The most notable changes consisted in th e overexpression of ErbB2 and ErbB3 by SCs of nerves from CMT1A patients. These findings are consistent with an impairment of SC differentiation and expand the molecular phenotype of CMT1 A. The upregulation of these receptors may play a ro le in the inhibition of myelination or in the promotion of recurrent demyelinati on and axonal damage. | Massa R. Palumbo C. Cavallaro T. 牛亚利 | 2006 | 世界核心医学期刊文摘(神经病学分册)2006，2，6： | 0 |
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