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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
1
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pubmed:dateCreated |
1983-7-8
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pubmed:abstractText |
Structural and functional studies were performed on a dysfunctional C8 molecule present in the serum of two siblings and an unrelated individual. The C8 in these three sera exhibited a pattern of partial immunologic identity with C8 in normal serum but was devoid of functional activity. The C8 was immunoprecipitated from the three sera and from a control serum with an antihuman C8 antiserum and analyzed by SDS-PAGE using highly purified human C8 as a reference. A selective absence of a band of 62,000 mol. wt was observed in the immunoprecipitates from the sera containing dysfunctional C8. Experiments performed with the purified alpha-gamma and beta subunits showed that the hemolytic activity of the C8 deficient sera could be reconstituted by the addition of the beta chain but not the alpha-gamma dimer. Binding of the dysfunctional C8 to C567 was excluded by the following observations: (1) EAC1-7 treated with the C8 deficient sera and then washed could not be lysed after the addition of the beta subunit and C9; and (2) the abnormal molecules did not interfere with the consumption of normal C8 by the soluble complex SC5b-7.
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pubmed:grant | |
pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Jan
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pubmed:issn |
0161-5890
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
20
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
47-51
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pubmed:dateRevised |
2007-11-14
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pubmed:meshHeading | |
pubmed:year |
1983
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pubmed:articleTitle |
Beta chain deficiency in three patients with dysfunctional C8 molecules.
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.
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