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Light and electron microscopy of the SD and the GM1 retinas revealed storage in ganglion cells. Similar to previous findings in rat retina, GD3 was the major ganglioside in mouse retina, while GM2 and GM1 were minor species. Total ganglioside content was 44% and 40% higher in the SD and the GM1 retinas, respectively, than in the control retinas. Furthermore, GM2 and GM1 content were 11\u2010fold and 51\u2010fold higher in the SD and the GM1 retinas than in the control retinas, respectively. High concentrations of asialo\u2010GM2 and asialo\u2010GM1 were found in the SD and the GM1 retinas, respectively, but were undetectable in the control retinas. The GSL abnormalities in the SD and the GM1 retinas reflect significant reductions in \u03b2\u2010hexosaminidase and \u03b2\u2010galactosidase enzyme activities, respectively. Although electroretinograms appeared normal in the SD and the GM1 mice, visual evoked potentials were subnormal in both mutants, indicating visual impairments. Our findings present a model system for assessing retinal pathobiology and therapies for the gangliosidoses.<\/jats:p>","DOI":"10.1111\/j.1471-4159.2007.04525.x","type":"journal-article","created":{"date-parts":[[2007,5,2]],"date-time":"2007-05-02T15:49:57Z","timestamp":1178120997000},"page":"1294-1302","source":"Crossref","is-referenced-by-count":25,"title":["Neurochemical, morphological, and neurophysiological abnormalities in retinas of Sandhoff and GM1 gangliosidosis mice"],"prefix":"10.1111","volume":"101","author":[{"given":"Christine A.","family":"Denny","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph","family":"Alroy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Basil S.","family":"Pawlyk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael A.","family":"Sandberg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandra","family":"D\u2019Azzo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas N.","family":"Seyfried","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2007,2,26]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02899544"},{"key":"e_1_2_7_3_1","first-page":"675","article-title":"Canine GM1\u2010gangliosidosis. 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