{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,25]],"date-time":"2025-09-25T16:44:45Z","timestamp":1758818685572},"reference-count":22,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2009,2,5]],"date-time":"2009-02-05T00:00:00Z","timestamp":1233792000000},"content-version":"vor","delay-in-days":3902,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["bpspubs.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["British J Pharmacology"],"published-print":{"date-parts":[[1998,6]]},"abstract":"<jats:p>\n<jats:list list-type=\"explicit-label\">\n<jats:list-item><jats:p>The progressive motor neuronopathy (<jats:italic>pmn<\/jats:italic>) mouse is an autosomal recessive mutant, in which the homozygotes suffer caudio\u2010cranial degeneration of motor axons and die several weeks after birth. This strain provides the opportunity of testing potential therapeutic strategies for the treatment of motor neurone diseases such as amyotrophic lateral sclerosis. We have performed a study of the effects on the <jats:italic>pmn<\/jats:italic> mouse of SR 57746A, an orally\u2010active, non\u2010peptide compound which has been found to exhibit neurotrophic effects <jats:italic>in vitro<\/jats:italic> and <jats:italic>in vivo<\/jats:italic>. In order to treat the affected mice from birth, the mothers were administered 2.5 mg kg<jats:sup>\u22121<\/jats:sup>, p.o., SR 57746A every two days until the weaning of the offspring (at day 20); then the offspring were given every two days a dose of 30 \u03bcg kg<jats:sup>\u22121<\/jats:sup>, p.o., until their death.<\/jats:p><\/jats:list-item>\n<jats:list-item><jats:p>Affected mice treated with SR 57746A had a lifespan 50% longer than that of the vehicle\u2010treated mice (<jats:italic>P<\/jats:italic>=0.01). Compared to vehicle\u2010treated <jats:italic>pmn<\/jats:italic> mice, SR 57746A improved the performance of the <jats:italic>pmn<\/jats:italic> mice in three different behavioural tasks. SR 57746A also maintained the amplitude of the motor evoked response of the gastrocnemius muscle, reduced the distal motor latency, and delayed the occurrence of the spontaneous denervation activity in this muscle. Histological studies indicated that at 20 days of age the mean surface areas of the fibres of the sciatic nerve were higher in SR 57746A\u2010treated than in vehicle\u2010treated mice.<\/jats:p><\/jats:list-item>\n<jats:list-item><jats:p>At present, SR 57746A is the only orally active, nonpeptide compound known to be capable of delaying the progression of the motor neurone degeneration in <jats:italic>pmn<\/jats:italic> mice.<\/jats:p><\/jats:list-item>\n<\/jats:list>\n<\/jats:p>","DOI":"10.1038\/sj.bjp.0701885","type":"journal-article","created":{"date-parts":[[2005,1,28]],"date-time":"2005-01-28T19:30:35Z","timestamp":1106940635000},"page":"811-817","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["The effect of the nonpeptide neurotrophic compound SR 57746A on the progression of the disease state of the <i>pmn<\/i> mouse"],"prefix":"10.1111","volume":"124","author":[{"given":"F","family":"Duong","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J","family":"Fournier","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P E","family":"Keane","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J L","family":"Gu\u00e9net","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P","family":"Soubri\u00e9","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J M","family":"Warter","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J","family":"Borg","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P","family":"Poindron","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2009,2,5]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/367368a0"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/0896-6273(95)90024-1"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/0022-510X(95)00315-S"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880140704"},{"key":"e_1_2_5_6_1","first-page":"120P","article-title":"Neurotrophic effects of SR 57746A, a new non\u2010peptide compound: in vitro studies","volume":"106","author":"Fournier J.","year":"1992","journal-title":"Br. 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