{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,16]],"date-time":"2025-10-16T20:00:15Z","timestamp":1760644815782},"reference-count":41,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2004,6,16]],"date-time":"2004-06-16T00:00:00Z","timestamp":1087344000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Neuroscience Research"],"published-print":{"date-parts":[[2004,8,15]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The <jats:italic>twitcher<\/jats:italic> mouse is well known as a naturally occurring authentic mouse model of human globoid cell leukodystrophy (GLD; Krabbe disease) due to genetic deficiency of lysosomal galactosylceramidase. The <jats:italic>twitcher<\/jats:italic> mice used most commonly are on the C57BL\/6J background. We generated <jats:italic>twitcher<\/jats:italic> mice that were on the mixed background of C57BL\/6J and 129SvEv, the standard strain for production of targeted mutations. <jats:italic>Twitcher<\/jats:italic> mice on the mixed background were smaller and had a shorter lifespan than were those on the C57BL\/6J background. Many <jats:italic>twitcher<\/jats:italic> mice on the mixed background developed generalized seizures around 30 days that were never seen in <jats:italic>twitcher<\/jats:italic> mice on the C57BL\/6J background. Neuropathologically, although the degree of the typical demyelination with infiltration of macrophages was similar in the central and peripheral nervous systems, in both strains, marked neuronal cell death was observed only in <jats:italic>twitcher<\/jats:italic> mice on the mixed background. In the hippocampus, the neuronal cell death occurred prominently in the CA3 region in contrast to the relatively well\u2010preserved CA1 and CA2 areas. This neuropathology has never been seen in <jats:italic>twitcher<\/jats:italic> mice on the C57BL\/6J background. Biochemically, the brain of <jats:italic>twitcher<\/jats:italic> mice on the mixed background showed much greater accumulation of lactosylceramide. Genetic background must be carefully taken into consideration when phenotype of mutant mice is evaluated, particularly because most targeted mutants are initially on a mixed genetic background and gradually moved to a pure background. These findings also suggest an intriguing possibility of important function of some sphingolipids in the hippocampal neuronal organization and maintenance. \u00a9 2004 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/jnr.20190","type":"journal-article","created":{"date-parts":[[2004,6,16]],"date-time":"2004-06-16T19:45:28Z","timestamp":1087415128000},"page":"507-516","source":"Crossref","is-referenced-by-count":24,"title":["Genetic background markedly influences vulnerability of the hippocampal neuronal organization in the \u201c<i>twitcher<\/i>\u201d mouse model of globoid cell leukodystrophy"],"prefix":"10.1002","volume":"77","author":[{"given":"Kumiko","family":"Tominaga","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Junko","family":"Matsuda","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Makiko","family":"Kido","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Etsuo","family":"Naito","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ichiro","family":"Yokota","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kazunori","family":"Toida","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kazunori","family":"Ishimura","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kunihiko","family":"Suzuki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasuhiro","family":"Kuroda","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2004,6,16]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"crossref","first-page":"2389","DOI":"10.1016\/S0021-9258(18)97335-7","article-title":"Study 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