rdf:type |
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lifeskim:mentions |
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pubmed:issue |
3
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pubmed:dateCreated |
1990-10-11
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pubmed:abstractText |
The sera from three C8 alpha-gamma deficient patients previously reported to have a selective C8 alpha-gamma defect were analyzed by SDS-PAGE and Western blot using two polyclonal antisera to C8 alpha-gamma and a monoclonal antibody to C8 alpha. All three sera exhibited C8 alpha-gamma bands that dissociated into alpha and gamma chains under reducing conditions. Quantitation of the alpha-gamma subunit in these sera by a sensitive ELISA revealed an amount approximately 1% of that found in normal human serum. A similar assay performed with a specific antiserum to C8 beta showed unexpectedly low levels of C8 beta in these sera, which were confirmed by hemolytic titration of C8 beta. The remarkable differences between C8 alpha-gamma and C8 beta in the C8 alpha-gamma deficient sera was that in spite of their comparable immunochemical levels, C8 beta still exhibited functional activity whereas C8 alpha-gamma was totally inactive. That the residual C8 alpha-gamma was inactive was also proved by its inability to show lytic bands in an overlay system after SDS-PAGE and subsequent removal of SDS. The implications of these findings for a novel concept of C8 deficiency are discussed.
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pubmed:grant |
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pubmed:commentsCorrections |
http://linkedlifedata.com/resource/pubmed/commentcorrection/2394837-2459199,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2394837-2613233,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2394837-2644073,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2394837-2656079,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2394837-3171181,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2394837-3312411,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2394837-3562470,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2394837-3685247,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2394837-3798025,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2394837-5432063,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2394837-558524,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2394837-6415112,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2394837-6433145,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2394837-6544189,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2394837-6822660,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2394837-6833775,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2394837-7379355,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2394837-7440581,
http://linkedlifedata.com/resource/pubmed/commentcorrection/2394837-815273
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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 |
Sep
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pubmed:issn |
0021-9738
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:volume |
86
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
884-8
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pubmed:dateRevised |
2009-11-18
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pubmed:meshHeading |
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pubmed:year |
1990
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pubmed:articleTitle |
Two distinct abnormalities in patients with C8 alpha-gamma deficiency. Low level of C8 beta chain and presence of dysfunctional C8 alpha-gamma subunit.
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pubmed:affiliation |
Istituto di Patologia Generale, Università di Trieste, Italy.
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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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