Department of Surgical Sciences, School of Veterinary Medicine, University of Wisconsin-Madison, 2015 Linden Drive, Madison, WI 53706, USA.
Department of Comparative Biosciences, School of Veterinary Medicine, University of Wisconsin-Madison, 2015 Linden Drive, Madison, WI 53706, USA.
Exp Neurol. 2023 Oct;368:114520. doi: 10.1016/j.expneurol.2023.114520. Epub 2023 Aug 25.
Wobbly hedgehog syndrome (WHS) has been long considered to be a myelin disease primarily affecting the four-toed hedgehog. In this study, we have shown for the first time that demyelination is accompanied by extensive remyelination in WHS. However, remyelination is not enough to compensate for the axonal degeneration and neuronal loss, resulting in a progressive neurodegenerative disease reminiscent of progressive forms of multiple sclerosis (MS) in humans. Thus, understanding the pathological features of WHS may shed light on the disease progression in progressive MS and ultimately help to develop therapeutic strategies for both diseases.
棘球蚴病（WHS）长期以来一直被认为是一种主要影响四趾刺猬的髓鞘疾病。在这项研究中，我们首次表明脱髓鞘伴有广泛的髓鞘再生。然而，髓鞘再生不足以补偿轴突变性和神经元丢失，导致进行性神经退行性疾病，使人联想到人类的进行性多发性硬化症（MS）形式。因此，了解 WHS 的病理特征可能有助于阐明进行性 MS 的疾病进展，并最终有助于为这两种疾病开发治疗策略。
