Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
3
pubmed:dateCreated
1990-10-11
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.
pubmed:grant
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
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
AIM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Sep
pubmed:issn
0021-9738
pubmed:author
pubmed:issnType
Print
pubmed:volume
86
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
884-8
pubmed:dateRevised
2009-11-18
pubmed:meshHeading
pubmed:year
1990
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.
pubmed:affiliation
Istituto di Patologia Generale, Università di Trieste, Italy.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S., Research Support, Non-U.S. Gov't