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Biomedical subjects

G Hauptmann

Publications and source records attributed to G Hauptmann.

150 records · Page 9Linked to original sources

Partial deficiency of the fourth component of human complement (C4) and autoantibody directed against C4 in a patient with SLE.

The finding of dramatically depressed levels of C4 in a 17-year old patient with severe systemic lupus erythematosus (SLE) prompted a genetic study of her family. This study revealed the existence of a partial C4 deficiency; we found the presence of a C4A3,C4BQo haplotype which was transmitted by the mother to each of her children. This patient possessed, in her serum, an autoantibody with anti-C4 specificity. The immunochemical characterization of this autoantibody revealed that it was IgM and belonged to the immunoconglutinin family. We have studied the effects of this autoantibody on the formation and dissociation kinetics of classical C3-convertase.

Adolescent↗

[Plasma and synovial fibronectin. Results of personal research].

The authors report the results of an assay of plasma fibronectin and synovial fluid fibronectin conducted in 104 patients with arthropathy, including 26 cases of rheumatoid arthritis, 43 cases of mechanical arthropathy and 35 cases of non-rheumatoid arthritis. The plasma fibronectin levels do not vary significantly between the different groups of patients, but the mean value for the whole group of patients is significantly lower than that of a control population. The mean value of synovial fluid fibronectin was significantly higher in the patients with rheumatoid arthritis (583 +/- 76 mg/1) than in the patients with non-rheumatoid arthritis (379 +/- 58 mg/1) or mechanical arthropathy (367 +/- 32 mg/1). The assay of synovial fibronectin could therefore provide useful information for the aetiological diagnosis of inflammatory arthropathies. The local origin, from the synovial membrane itself, of the fibronectin in the synovial fluid is suggested by the fact that the synovial concentration of this protein is superior to the serum concentration in 50 to 90 per cent of cases, depending on the disease.

Arthritis↗

[Genetics of complement: recent aspects (author's transl)].

Genetic deficiencies of complement proteins are now more often recognized and analysed more precisely because the structure of the different complement proteins is better known. Partial defects may be detected in some components by the combined utilization of titration techniques, polymorphism studies and linkage analyses. The partial deficiency in C4 seems to be the most frequent protein deficiency in the human. The complement markers on the short arm of the sixth chromosome in man (BF, C2, C4A and C4B) are located in close proximity to the HLA-D/DR region. The combined study of the complement and HLA markers will probably allow the fine structure of the HLA region to be better defined. The association of some diseases with HLA types will probably also be better specified by the definition of associations not only with HLA-B or HLA-D types but also with the BF, C2 and C4 types.

Animals↗