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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
6
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pubmed:dateCreated |
1976-2-20
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pubmed:abstractText |
The residual beta-D-galactosidase activity (10% of normal) present in an autopsy sample of liver derived from an I-cell patient has been characterized. The pH optima for both I-cell and normal acid 4-methylumbelliferyl beta-D-galactoside activities were 4.35. The adsorption and elution profiles of the I-cell enzyme from Con A-Sepharose were similar to those of normal liver beta-D-galactosidase. Although starch gel electrophoresis revealed the presence of beta-D-galactosidase A and B in I-cell disease liver, the A band was more diffuse and migrated less anodally than the A band from normal liver. The electrophoretic mobilities of both I-cell and normal beta-D-galactosidase A appeared to decrease after treatment with neuraminidase. Kinetic studies of the I-cell and normal level beta-D-galactosidase demonstrated similar apparent Km values with respect to the 4-methylumbelliferyl beta-D-galactoside and Gm1 ganglioside, whereas the Vmax values obtained for the I-cell enzyme were 10- to 12-fold lower than those of the normal enzyme for both substrates.
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pubmed:commentsCorrections |
http://linkedlifedata.com/resource/pubmed/commentcorrection/812360-13638269,
http://linkedlifedata.com/resource/pubmed/commentcorrection/812360-14907713,
http://linkedlifedata.com/resource/pubmed/commentcorrection/812360-17842782,
http://linkedlifedata.com/resource/pubmed/commentcorrection/812360-4100420,
http://linkedlifedata.com/resource/pubmed/commentcorrection/812360-4192208,
http://linkedlifedata.com/resource/pubmed/commentcorrection/812360-4200723,
http://linkedlifedata.com/resource/pubmed/commentcorrection/812360-4327936,
http://linkedlifedata.com/resource/pubmed/commentcorrection/812360-4327937,
http://linkedlifedata.com/resource/pubmed/commentcorrection/812360-4345092,
http://linkedlifedata.com/resource/pubmed/commentcorrection/812360-4346288,
http://linkedlifedata.com/resource/pubmed/commentcorrection/812360-4359899,
http://linkedlifedata.com/resource/pubmed/commentcorrection/812360-4364008,
http://linkedlifedata.com/resource/pubmed/commentcorrection/812360-4708053,
http://linkedlifedata.com/resource/pubmed/commentcorrection/812360-4784465,
http://linkedlifedata.com/resource/pubmed/commentcorrection/812360-4823437,
http://linkedlifedata.com/resource/pubmed/commentcorrection/812360-4881884,
http://linkedlifedata.com/resource/pubmed/commentcorrection/812360-5096955,
http://linkedlifedata.com/resource/pubmed/commentcorrection/812360-5567403
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
AIM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:month |
Nov
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pubmed:issn |
0002-9297
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:volume |
27
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
719-27
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pubmed:dateRevised |
2010-9-3
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pubmed:meshHeading |
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pubmed:year |
1975
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pubmed:articleTitle |
Characterization of beta-D-galactosidase isolated from I-cell disease liver.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.
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