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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منابع مشابه
Occurrence of an incomplete C8 molecule in homozygous C8 deficiency in man
Sera from unrelated individuals with recurrent Neisserial infections lacked C8 hemolytic activity, but contained a protein that is antigenically related to C8. Immunochemical analysis revealed complete identity of the C8-related protein of all three sera and a marked antigenic deficiency compared with normal C8. The C8-related protein was isolated from serum by adsorption to immobilized anti-C8...
متن کاملCorrection: Regioselective C8-metalation of N-phosphine tethered adenine derivatives via C8-H activation.
Correction for 'Regioselective C8-metalation of N-phosphine tethered adenine derivatives via C8-H activation' by D. Brackemeyer et al., Dalton Trans., 2017, DOI: 10.1039/c7dt00682a.
متن کاملThe preparation and characterization of monoclonal antibodies to human complement component C8 and their use in purification of C8 and C8 subunits.
1. Ten mouse monoclonal antibodies to human complement component C8 were prepared. It was found that six of these antibodies reacted with the alpha-subunit, two with the beta-subunit and two with the gamma-subunit, when assessed by immunoblotting after separation of C8 subunits by SDS/polyacrylamide-gel electrophoresis. 2. Epitope analysis of the ten monoclonal antibodies in a competitive bindi...
متن کاملOxidation of Short-Chain Fatty Acids (C1-C8)
Gomori, K. (1943). J. biol. Chem. 148, 139. Harris, W. D. & Popat, P. (1954). J. Amer. Oil Chem. Soc. 81, 124. Hers, H. G. (1957). Le Matabolieme due Fructo8e, p. 95. Brussels: lditions Arscia. Lohman, K. (1933). Biochem. Z. 262, 137. McKrasch, L. C., Davidson, W. P. & McGilvery, R. W. (1956). J. biol. Chem. 222,179. McKrasch, L. C. & McGilvery, R. W. (1956). J. biol. Chem. 221, 909. Newsholme,...
متن کاملRegioselective C8-metalation of N-phosphine tethered adenine derivatives via C8-H activation.
Two new adenine derivatives bearing a N-methylene (1) or N-ethylene (2) tethered diphenylphosphine group have been reacted with [MIIICl2Cp*]2 (M = Ir, Rh) or [RuIIClCp*]4. The formation of Werner-type phosphine complexes [3]-[6] and upon heating their subsequent rearrangement into complexes bearing an NH,NR-NHC^PRPh2 chelate ligand in [7]Cl (M = Ir) and [8] (M = Ru) have been observed.
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ژورنال
عنوان ژورنال: Journal of Clinical Investigation
سال: 1990
ISSN: 0021-9738
DOI: 10.1172/jci114789