RP-CARS揭示了Krabbe病动物模型髓鞘中的分子空间秩序异常
RP-CARS reveals molecular spatial order anomalies in myelin of an animal model of Krabbe disease
摘要：
Krabbe病（KD）是一种罕见的脱髓鞘性鞘脂病，通常在生命的最初几年内致命。它是由半乳糖苷酶（GALC）失活引起的，该酶导致被认为是组织水平效应起源的精神病细胞水平增加。GALC也在Twitch（TWI）小鼠中失活：KD的遗传模型，为理解发病过程和开发可能的治疗方法提供了重要的见解。在这篇文章中，一个创新的光学技术，RP CARS，提出了作为一种工具来研究的程度的有序的CH 2键内的髓鞘的TWI-小鼠坐骨神经纤维。RP-CARS是近年来发展起来的一种CARS显微技术，它将CARS显微技术固有的化学选择性与分子键空间取向灵敏度结合起来。这是RP-CARS首次应用于病理学遗传模型的研究，从而证明了髓鞘CH 2键的发病后进行性空间解体。所提出的结果可能是一个更深入地了解人类KD的致病机制的巨大兴趣，而且，它是这种显微镜技术的实验有效性的一个额外的证明。
英文摘要：
Krabbe disease (KD) is a rare demyelinating sphingolipidosis, often fatal in the first years of life. It is caused by the inactivation of the galactocerebrosidase (GALC) enzyme that causes an increase in the cellular levels of psychosine considered to be at the origin of the tissue-level effects. GALC is inactivated also in the Twitcher (TWI) mouse: a genetic model of KD that is providing important insights into the understating of the pathogenetic process and the development of possible treatments. In this article an innovative optical technique, RP-CARS, is proposed as a tool to study the degree of order of the CH2 bonds inside the myelin sheaths of TWI-mice sciatic-nerve fibres. RP-CARS, a recently developed variation of CARS microscopy, is able to combine the intrinsic chemical selectivity of CARS microscopy with molecular-bond-spatial-orientation sensibility. This is the first time RP-CARS is applied to the study of a genetic model of a pathology, leading to the demonstration of a post-onset progressive spatial disorganization of the myelin CH2 bonds. The presented result could be of great interest for a deeper understanding of the pathogenic mechanisms underlying the human KD and, moreover, it is an additional proof of the experimental validity of this microscopy technique.
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