Therapeutic evaluation of GM2 gangliosidoses by ELISA using anti-GM2 ganglioside antibodies
GM2 gangliosidoses, including Tay-Sachs disease, Sandhoff disease and the AB variant, comprise deficiencies of β-hexosaminidase isozymes and GM2 ganglioside activator protein associated with accumulation of GM2 ganglioside (GM2) in lysosomes and neurosomatic clinical manifestations. A simple assay s...
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Veröffentlicht in: | Clinica chimica acta 2007-03, Vol.378 (1), p.38-41 |
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creator | Tsuji, Daisuke Higashine, Yukari Matsuoka, Kazuhiko Sakuraba, Hitoshi Itoh, Kohji |
description | GM2 gangliosidoses, including Tay-Sachs disease, Sandhoff disease and the AB variant, comprise deficiencies of β-hexosaminidase isozymes and GM2 ganglioside activator protein associated with accumulation of GM2 ganglioside (GM2) in lysosomes and neurosomatic clinical manifestations. A simple assay system for intracellular quantification of GM2 is required to evaluate the therapeutic effects on GM2-gangliosidoses.
We newly established a cell–ELISA system involving anti-GM2 monoclonal antibodies for measuring GM2 storage in fibroblasts from Tay-Sachs and Sandhoff disease patients.
We succeeded in detecting the corrective effect of enzyme replacement on elimination of GM2 in the cells with this ELISA system.
This simple and sensitive system should be useful as additional diagnosis tool as well as therapeutic evaluation of GM2 gangliosidoses. |
doi_str_mv | 10.1016/j.cca.2006.10.010 |
format | Article |
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We newly established a cell–ELISA system involving anti-GM2 monoclonal antibodies for measuring GM2 storage in fibroblasts from Tay-Sachs and Sandhoff disease patients.
We succeeded in detecting the corrective effect of enzyme replacement on elimination of GM2 in the cells with this ELISA system.
This simple and sensitive system should be useful as additional diagnosis tool as well as therapeutic evaluation of GM2 gangliosidoses.</description><identifier>ISSN: 0009-8981</identifier><identifier>EISSN: 1873-3492</identifier><identifier>DOI: 10.1016/j.cca.2006.10.010</identifier><identifier>PMID: 17196574</identifier><language>eng</language><publisher>Netherlands: Elsevier B.V</publisher><subject>Antibodies, Monoclonal - therapeutic use ; beta-Hexosaminidase beta Chain ; beta-N-Acetylhexosaminidases - therapeutic use ; Cells, Cultured ; Cell–ELISA ; Diagnosis ; Enzyme replacement therapy ; Enzyme-Linked Immunosorbent Assay - methods ; Fibroblasts - chemistry ; G(M2) Ganglioside - analysis ; G(M2) Ganglioside - immunology ; Gangliosidoses, GM2 - therapy ; GM2 ganglioside ; Hexosaminidase B ; Humans ; Sandhoff disease ; Sandhoff Disease - therapy ; Tay-Sachs disease ; Tay-Sachs Disease - therapy</subject><ispartof>Clinica chimica acta, 2007-03, Vol.378 (1), p.38-41</ispartof><rights>2006</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c417t-6147f82dd9b2b6d21d0c25b78b605e020dd2efeb758077845d0dee38f59e30353</citedby><cites>FETCH-LOGICAL-c417t-6147f82dd9b2b6d21d0c25b78b605e020dd2efeb758077845d0dee38f59e30353</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/j.cca.2006.10.010$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3550,27924,27925,45995</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/17196574$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Tsuji, Daisuke</creatorcontrib><creatorcontrib>Higashine, Yukari</creatorcontrib><creatorcontrib>Matsuoka, Kazuhiko</creatorcontrib><creatorcontrib>Sakuraba, Hitoshi</creatorcontrib><creatorcontrib>Itoh, Kohji</creatorcontrib><title>Therapeutic evaluation of GM2 gangliosidoses by ELISA using anti-GM2 ganglioside antibodies</title><title>Clinica chimica acta</title><addtitle>Clin Chim Acta</addtitle><description>GM2 gangliosidoses, including Tay-Sachs disease, Sandhoff disease and the AB variant, comprise deficiencies of β-hexosaminidase isozymes and GM2 ganglioside activator protein associated with accumulation of GM2 ganglioside (GM2) in lysosomes and neurosomatic clinical manifestations. A simple assay system for intracellular quantification of GM2 is required to evaluate the therapeutic effects on GM2-gangliosidoses.
We newly established a cell–ELISA system involving anti-GM2 monoclonal antibodies for measuring GM2 storage in fibroblasts from Tay-Sachs and Sandhoff disease patients.
We succeeded in detecting the corrective effect of enzyme replacement on elimination of GM2 in the cells with this ELISA system.
This simple and sensitive system should be useful as additional diagnosis tool as well as therapeutic evaluation of GM2 gangliosidoses.</description><subject>Antibodies, Monoclonal - therapeutic use</subject><subject>beta-Hexosaminidase beta Chain</subject><subject>beta-N-Acetylhexosaminidases - therapeutic use</subject><subject>Cells, Cultured</subject><subject>Cell–ELISA</subject><subject>Diagnosis</subject><subject>Enzyme replacement therapy</subject><subject>Enzyme-Linked Immunosorbent Assay - methods</subject><subject>Fibroblasts - chemistry</subject><subject>G(M2) Ganglioside - analysis</subject><subject>G(M2) Ganglioside - immunology</subject><subject>Gangliosidoses, GM2 - therapy</subject><subject>GM2 ganglioside</subject><subject>Hexosaminidase B</subject><subject>Humans</subject><subject>Sandhoff disease</subject><subject>Sandhoff Disease - therapy</subject><subject>Tay-Sachs disease</subject><subject>Tay-Sachs Disease - therapy</subject><issn>0009-8981</issn><issn>1873-3492</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2007</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kD1PwzAQhi0EoqXwA1hQJraEs_NhR0xVVUqlIgbKxGA59qW4SuMSJ5X670lpJcTCdLrT877SPYTcUogo0OxhHWmtIgaQ9XsEFM7IkAoeh3GSs3MyBIA8FLmgA3Ll_bpfE8joJRlQTvMs5cmQfCw_sVFb7FqrA9ypqlOtdXXgymD2woKVqleVdd4a59EHxT6YLuZv46Dztl4Fqm5t-BfDn2PhjEV_TS5KVXm8Oc0ReX-aLifP4eJ1Np-MF6FOKG_DjCa8FMyYvGBFZhg1oFlacFFkkCIwMIZhiQVPBXAuktSAQYxFmeYYQ5zGI3J_7N027qtD38qN9RqrStXoOi-zHCgXNO9BegR147xvsJTbxm5Us5cU5MGoXMveqDwYPZx6o33m7lTeFRs0v4mTwh54PALYv7iz2EivLdYajW1Qt9I4-0_9N_GkhbY</recordid><startdate>20070301</startdate><enddate>20070301</enddate><creator>Tsuji, Daisuke</creator><creator>Higashine, Yukari</creator><creator>Matsuoka, Kazuhiko</creator><creator>Sakuraba, Hitoshi</creator><creator>Itoh, Kohji</creator><general>Elsevier B.V</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20070301</creationdate><title>Therapeutic evaluation of GM2 gangliosidoses by ELISA using anti-GM2 ganglioside antibodies</title><author>Tsuji, Daisuke ; Higashine, Yukari ; Matsuoka, Kazuhiko ; Sakuraba, Hitoshi ; Itoh, Kohji</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c417t-6147f82dd9b2b6d21d0c25b78b605e020dd2efeb758077845d0dee38f59e30353</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2007</creationdate><topic>Antibodies, Monoclonal - therapeutic use</topic><topic>beta-Hexosaminidase beta Chain</topic><topic>beta-N-Acetylhexosaminidases - therapeutic use</topic><topic>Cells, Cultured</topic><topic>Cell–ELISA</topic><topic>Diagnosis</topic><topic>Enzyme replacement therapy</topic><topic>Enzyme-Linked Immunosorbent Assay - methods</topic><topic>Fibroblasts - chemistry</topic><topic>G(M2) Ganglioside - analysis</topic><topic>G(M2) Ganglioside - immunology</topic><topic>Gangliosidoses, GM2 - therapy</topic><topic>GM2 ganglioside</topic><topic>Hexosaminidase B</topic><topic>Humans</topic><topic>Sandhoff disease</topic><topic>Sandhoff Disease - therapy</topic><topic>Tay-Sachs disease</topic><topic>Tay-Sachs Disease - therapy</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Tsuji, Daisuke</creatorcontrib><creatorcontrib>Higashine, Yukari</creatorcontrib><creatorcontrib>Matsuoka, Kazuhiko</creatorcontrib><creatorcontrib>Sakuraba, Hitoshi</creatorcontrib><creatorcontrib>Itoh, Kohji</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Clinica chimica acta</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Tsuji, Daisuke</au><au>Higashine, Yukari</au><au>Matsuoka, Kazuhiko</au><au>Sakuraba, Hitoshi</au><au>Itoh, Kohji</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Therapeutic evaluation of GM2 gangliosidoses by ELISA using anti-GM2 ganglioside antibodies</atitle><jtitle>Clinica chimica acta</jtitle><addtitle>Clin Chim Acta</addtitle><date>2007-03-01</date><risdate>2007</risdate><volume>378</volume><issue>1</issue><spage>38</spage><epage>41</epage><pages>38-41</pages><issn>0009-8981</issn><eissn>1873-3492</eissn><abstract>GM2 gangliosidoses, including Tay-Sachs disease, Sandhoff disease and the AB variant, comprise deficiencies of β-hexosaminidase isozymes and GM2 ganglioside activator protein associated with accumulation of GM2 ganglioside (GM2) in lysosomes and neurosomatic clinical manifestations. A simple assay system for intracellular quantification of GM2 is required to evaluate the therapeutic effects on GM2-gangliosidoses.
We newly established a cell–ELISA system involving anti-GM2 monoclonal antibodies for measuring GM2 storage in fibroblasts from Tay-Sachs and Sandhoff disease patients.
We succeeded in detecting the corrective effect of enzyme replacement on elimination of GM2 in the cells with this ELISA system.
This simple and sensitive system should be useful as additional diagnosis tool as well as therapeutic evaluation of GM2 gangliosidoses.</abstract><cop>Netherlands</cop><pub>Elsevier B.V</pub><pmid>17196574</pmid><doi>10.1016/j.cca.2006.10.010</doi><tpages>4</tpages></addata></record> |
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subjects | Antibodies, Monoclonal - therapeutic use beta-Hexosaminidase beta Chain beta-N-Acetylhexosaminidases - therapeutic use Cells, Cultured Cell–ELISA Diagnosis Enzyme replacement therapy Enzyme-Linked Immunosorbent Assay - methods Fibroblasts - chemistry G(M2) Ganglioside - analysis G(M2) Ganglioside - immunology Gangliosidoses, GM2 - therapy GM2 ganglioside Hexosaminidase B Humans Sandhoff disease Sandhoff Disease - therapy Tay-Sachs disease Tay-Sachs Disease - therapy |
title | Therapeutic evaluation of GM2 gangliosidoses by ELISA using anti-GM2 ganglioside antibodies |
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