Biochemical heterogeneity of infantile central nervous system spongy degeneration
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 f...
Saved in:
| Main Author: | |
|---|---|
| Other Authors: | , |
| Format: | article |
| Published: |
1992
|
| Online Access: | http://hdl.handle.net/10725/6530 http://libraries.lau.edu.lb/research/laur/terms-of-use/articles.php http://journals.sagepub.com/doi/abs/10.1177/08830738920070010411 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1864513480185348096 |
|---|---|
| author | Youssef, Nazih |
| author2 | Tipirnemi, Aparna Subramanyan, Sarvepalli B. |
| author2_role | author author |
| author_facet | Youssef, Nazih Tipirnemi, Aparna Subramanyan, Sarvepalli B. |
| author_role | author |
| dc.creator.none.fl_str_mv | Youssef, Nazih Tipirnemi, Aparna Subramanyan, Sarvepalli B. |
| dc.date.none.fl_str_mv | 1992 2017-11-07T13:46:53Z 2017-11-07T13:46:53Z 2017-11-07 |
| dc.identifier.none.fl_str_mv | 1708-8283 http://hdl.handle.net/10725/6530 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. http://libraries.lau.edu.lb/research/laur/terms-of-use/articles.php http://journals.sagepub.com/doi/abs/10.1177/08830738920070010411 |
| dc.language.none.fl_str_mv | en |
| dc.relation.none.fl_str_mv | Journal of Child Neurology |
| dc.rights.*.fl_str_mv | info:eu-repo/semantics/openAccess |
| dc.title.none.fl_str_mv | Biochemical heterogeneity of infantile central nervous system spongy degeneration |
| dc.type.none.fl_str_mv | Article info:eu-repo/semantics/publishedVersion info:eu-repo/semantics/article |
| description | 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 |
| eu_rights_str_mv | openAccess |
| format | article |
| id | LAURepo_563f385aa6b361364168038348fa6602 |
| identifier_str_mv | 1708-8283 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. |
| language_invalid_str_mv | en |
| network_acronym_str | LAURepo |
| network_name_str | Lebanese American University repository |
| oai_identifier_str | oai:laur.lau.edu.lb:10725/6530 |
| publishDate | 1992 |
| repository.mail.fl_str_mv | |
| repository.name.fl_str_mv | |
| repository_id_str | |
| spelling | Biochemical heterogeneity of infantile central nervous system spongy degenerationYoussef, NazihTipirnemi, AparnaSubramanyan, Sarvepalli B.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 degenerationPublishedN/A2017-11-07T13:46:53Z2017-11-07T13:46:53Z19922017-11-07Articleinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/article1708-8283http://hdl.handle.net/10725/6530Subramanyam, 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.http://libraries.lau.edu.lb/research/laur/terms-of-use/articles.phphttp://journals.sagepub.com/doi/abs/10.1177/08830738920070010411enJournal of Child Neurologyinfo:eu-repo/semantics/openAccessoai:laur.lau.edu.lb:10725/65302021-03-19T10:03:27Z |
| spellingShingle | Biochemical heterogeneity of infantile central nervous system spongy degeneration Youssef, Nazih |
| status_str | publishedVersion |
| title | Biochemical heterogeneity of infantile central nervous system spongy degeneration |
| title_full | Biochemical heterogeneity of infantile central nervous system spongy degeneration |
| title_fullStr | Biochemical heterogeneity of infantile central nervous system spongy degeneration |
| title_full_unstemmed | Biochemical heterogeneity of infantile central nervous system spongy degeneration |
| title_short | Biochemical heterogeneity of infantile central nervous system spongy degeneration |
| title_sort | Biochemical heterogeneity of infantile central nervous system spongy degeneration |
| url | http://hdl.handle.net/10725/6530 http://libraries.lau.edu.lb/research/laur/terms-of-use/articles.php http://journals.sagepub.com/doi/abs/10.1177/08830738920070010411 |