Biochemical heterogeneity of infantile central nervous system spongy degeneration

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dc.contributor.author Youssef, Nazih
dc.contributor.author Tipirnemi, Aparna
dc.contributor.author Subramanyan, Sarvepalli B.
dc.date.accessioned 2017-11-07T13:46:53Z
dc.date.available 2017-11-07T13:46:53Z
dc.date.copyright 1992 en_US
dc.date.issued 2017-11-07
dc.identifier.issn 1708-8283 en_US
dc.identifier.uri http://hdl.handle.net/10725/6530
dc.description.abstract Aspartoacylase, the enzyme whose activity is deficient in infantile central nervous system spongy degeneration (Canavan-Van Bogaert-Bertrand disease), is detected as an approximately 59-kD protein in the Sephadex G-200 filtration of normal fibroblast extracts. The enzyme activity in homogenates of fibroblasts is protected by leupeptin, a protease inhibitor. In the absence of leupeptin, 90% of aspartoacylase activity is lost. In some patients with infantile spongy degeneration, no activity (less than 2%) can be detected. In some other patients with residual activity in fibroblasts, two separate peaks of enzyme are eluted with molecular weight corresponding to approximately 59 and 19 kD. Aspartoacylase activity in this latter group is protected to the same extent by the presence of leupeptin. However, the elution of two peaks is independent of the presence of leupeptin. This study indicates biochemical heterogeneity in the pathogenesis of infantile spongy degeneration en_US
dc.language.iso en en_US
dc.title Biochemical heterogeneity of infantile central nervous system spongy degeneration en_US
dc.type Article en_US
dc.description.version Published en_US
dc.author.school SOM en_US
dc.author.idnumber 201005486 en_US
dc.author.department N/A en_US
dc.description.embargo N/A en_US
dc.relation.journal Journal of Child Neurology en_US
dc.journal.volume 7 en_US
dc.journal.issue 1 en_US
dc.article.pages S22-S25 en_US
dc.identifier.ctation Subramanyam, S. B., Tipirneni, A., Youssef, N., Gascon, G. G., & Ozand, P. T. (1992). Biochemical heterogeneity of infantile central nervous system spongy degeneration. Journal of child neurology, 7(1_suppl), S22-S25. en_US
dc.author.email nazih.youssef@lau.edu.lb en_US
dc.identifier.tou http://libraries.lau.edu.lb/research/laur/terms-of-use/articles.php en_US
dc.identifier.url http://journals.sagepub.com/doi/abs/10.1177/08830738920070010411 en_US
dc.author.affiliation Lebanese American University en_US

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