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rdf:type |
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lifeskim:mentions |
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pubmed:dateCreated |
1992-1-15
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pubmed:abstractText |
Metachromatic leukodystrophy is a lysosomal storage disease caused by the deficiency of arylsulphatase A (ASA). A human ASA cDNA was subcloned into the retroviral vector pXT1. Replication-defective retrovirus was generated by transfection of the vector into the amphotropic packaging cell line PA317. Human fibroblasts from a patient suffering from metachromatic leucodystrophy was infected with the recombinant retrovirus. Infected fibroblasts expressed ten times more ASA compared with control fibroblasts from a normal individual. The ASA encoded by the integrated provirus was shown to be correctly transported into the lysosomes and to normalize the impaired degradation of cerebroside sulphate.
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pubmed:commentsCorrections |
http://linkedlifedata.com/resource/pubmed/commentcorrection/1684103-1670590,
http://linkedlifedata.com/resource/pubmed/commentcorrection/1684103-1904721,
http://linkedlifedata.com/resource/pubmed/commentcorrection/1684103-1967188,
http://linkedlifedata.com/resource/pubmed/commentcorrection/1684103-1975241,
http://linkedlifedata.com/resource/pubmed/commentcorrection/1684103-2158095,
http://linkedlifedata.com/resource/pubmed/commentcorrection/1684103-2562955,
http://linkedlifedata.com/resource/pubmed/commentcorrection/1684103-2821499,
http://linkedlifedata.com/resource/pubmed/commentcorrection/1684103-3056714,
http://linkedlifedata.com/resource/pubmed/commentcorrection/1684103-3220255,
http://linkedlifedata.com/resource/pubmed/commentcorrection/1684103-3278379,
http://linkedlifedata.com/resource/pubmed/commentcorrection/1684103-3472751,
http://linkedlifedata.com/resource/pubmed/commentcorrection/1684103-3785217,
http://linkedlifedata.com/resource/pubmed/commentcorrection/1684103-4437317,
http://linkedlifedata.com/resource/pubmed/commentcorrection/1684103-6104322,
http://linkedlifedata.com/resource/pubmed/commentcorrection/1684103-6136972
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Dec
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pubmed:issn |
0264-6021
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:day |
1
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pubmed:volume |
280 ( Pt 2)
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
459-61
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pubmed:dateRevised |
2009-11-18
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pubmed:meshHeading |
pubmed-meshheading:1684103-Cell Fractionation,
pubmed-meshheading:1684103-Cell Line,
pubmed-meshheading:1684103-Cerebroside-Sulfatase,
pubmed-meshheading:1684103-Cerebrosides,
pubmed-meshheading:1684103-DNA,
pubmed-meshheading:1684103-Fibroblasts,
pubmed-meshheading:1684103-Humans,
pubmed-meshheading:1684103-Hydrolysis,
pubmed-meshheading:1684103-Leukodystrophy, Metachromatic,
pubmed-meshheading:1684103-Precipitin Tests,
pubmed-meshheading:1684103-Retroviridae,
pubmed-meshheading:1684103-Transfection
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pubmed:year |
1991
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pubmed:articleTitle |
Restoration of arylsulphatase A activity in human-metachromatic-leucodystrophy fibroblasts via retroviral-vector-mediated gene transfer.
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pubmed:affiliation |
Universität Göttingen, Abteilung Biochemie II, Federal Republic of Germany.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Research Support, Non-U.S. Gov't
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