{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T14:46:42Z","timestamp":1791298002954,"version":"4.1.0"},"reference-count":58,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2016,3,15]],"date-time":"2016-03-15T00:00:00Z","timestamp":1458000000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Biophotonics"],"published-print":{"date-parts":[[2017,3]]},"abstract":"<jats:p>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\u2010level 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\u2010CARS, is proposed as a tool to study the degree of order of the CH<jats:sub>2<\/jats:sub> bonds inside the myelin sheaths of TWI\u2010mice sciatic\u2010nerve fibres. RP\u2010CARS, a recently developed variation of CARS microscopy, is able to combine the intrinsic chemical selectivity of CARS microscopy with molecular\u2010bond\u2010spatial\u2010orientation sensibility. This is the first time RP\u2010CARS is applied to the study of a genetic model of a pathology, leading to the demonstration of a post\u2010onset progressive spatial disorganization of the myelin CH<jats:sub>2<\/jats:sub> 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.\n<jats:boxed-text content-type=\"graphic\" position=\"anchor\"><jats:caption><jats:p>RP\u2010CARS image (2850\u00a0cm<jats:sup>\u20131<\/jats:sup>, CH<jats:sub>2<\/jats:sub> bonds) of a sciatic\u2010nerve optical longitudinal section from a Twitcher P23 (symptomatic) mouse. Scale bar: 10\u00a0microns. The image was constructed by colour\u2010mapping the degree of molecular order of the CH<jats:sub>2<\/jats:sub> bonds inside the myelin walls, as displayed in the colour bar on the right.<\/jats:p><\/jats:caption><jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/jbio201500305-gra-0001-m.png\"><jats:alt-text>magnified image<\/jats:alt-text><jats:caption><jats:p>RP\u2010CARS image (2850\u00a0cm<jats:sup>\u20131<\/jats:sup>, CH<jats:sub>2<\/jats:sub> bonds) of a sciatic\u2010nerve optical longitudinal section from a Twitcher P23 (symptomatic) mouse. Scale bar: 10\u00a0microns. The image was constructed by colour\u2010mapping the degree of molecular order of the CH<jats:sub>2<\/jats:sub> bonds inside the myelin walls, as displayed in the colour bar on the right.<\/jats:p><\/jats:caption><\/jats:graphic><\/jats:boxed-text><\/jats:p>","DOI":"10.1002\/jbio.201500305","type":"journal-article","created":{"date-parts":[[2016,3,19]],"date-time":"2016-03-19T01:09:14Z","timestamp":1458349754000},"page":"385-393","source":"Crossref","is-referenced-by-count":18,"title":["RP\u2010CARS reveals molecular spatial order anomalies in myelin of an animal model of Krabbe disease"],"prefix":"10.1002","volume":"10","author":[{"given":"Giuseppe","family":"de Vito","sequence":"first","affiliation":[{"name":"NEST Scuola Normale Superiore  Piazza San Silvestro 12 I\u201056127 Pisa Italy"},{"name":"Center for Nanotechnology Innovation @NEST Istituto Italiano di Tecnologia  Piazza San Silvestro 12 I\u201056127 Pisa Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentina","family":"Cappello","sequence":"additional","affiliation":[{"name":"Center for Nanotechnology Innovation @NEST Istituto Italiano di Tecnologia  Piazza San Silvestro 12 I\u201056127 Pisa Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilaria","family":"Tonazzini","sequence":"additional","affiliation":[{"name":"NEST Scuola Normale Superiore and Istituto Nanoscienze\u2010CNR  Piazza San Silvestro 12 Pisa I\u201056127 Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Cecchini","sequence":"additional","affiliation":[{"name":"NEST Scuola Normale Superiore and Istituto Nanoscienze\u2010CNR  Piazza San Silvestro 12 Pisa I\u201056127 Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vincenzo","family":"Piazza","sequence":"additional","affiliation":[{"name":"Center for Nanotechnology Innovation @NEST Istituto Italiano di Tecnologia  Piazza San Silvestro 12 I\u201056127 Pisa Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,3,15]]},"reference":[{"key":"e_1_2_6_2_1","unstructured":"National Library of Medicine.Krabbe Disease. 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