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Biomedical subjects

F De Keyser

Publications and source records attributed to F De Keyser.

16 recordsLinked to original sources

Cross-reactivity of the B/B' subunit of the Sm ribonucleoprotein autoantigen with proline-rich polypeptides.

Using recombinant fusion proteins representing different regions of the human Sm B/B' polypeptide, the 4B4 monoclonal anti-Sm antibody was found to bind a C-terminus epitope that is proline-rich. 4B4 cross-reacted with the p24 gag protein of HIV-1 and with other polypeptides rich in proline residues, including collagen. BALB/c mice immunized with human collagen not only produced antibodies to the immunizing antigen but also antibodies to Sm. This immune mouse serum also recognized C-terminus B/B' fusion proteins. These data suggest that the Sm B/B' antigen contains a poly-Pro epitope that is shared by several autoantigens and retroviral proteins. These sites may be important in the induction of autoantibodies through molecular mimicry.

Animals

Crosstalk with external bipolar DVI pacing: a case report.

An increase of the basic atrial pacing rate from a preset value of 70 beats/min up to 91 beats/min was recorded in a patient with an external bipolar dual chamber pacing system. This observation could be explained by the occurrence of crosstalk; this specific manifestation of crosstalk was the result of the use of an uncommitted DVI pacing mode.

Cardiac Pacing, Artificial

Pulmonary metastases of a tibia adamantinoma. Case report and review of the literature.

An adamantinoma is a rare primary tumour of bone. It is usually seen by stomatologists as a tumour of the jaws, although several cases of the long bones, especially the tibia, have been described. The tumour is considered as a low grade malignancy with unknown histogenesis. It has a high rate of local recurrence after resection and metastasizes in part of the cases. This report describes a 41-year-old woman with multiple pulmonary metastases occurring 27 years after the diagnosis of a tibia adamantinoma. The clinical, radiographic and fiber-bronchoscopic findings are presented. The literature of adamantinoma metastasis is reviewed. The need for an early locally aggressive treatment is stressed as the treatment of metastatic disease seems very disappointing.

Adult

Onset of symptoms of rheumatoid arthritis in relation to age, sex and menopausal transition.

In a population of 564 patients with rheumatoid arthritis (RA), the onset of symptoms has been studied in relation to age, sex and last menstrual period for women. Median age of menopause was 49 years (3rd percentile: 32 years and 97th percentile: 56 years). Median age of first symptoms was 45 years in women and 50 in men. The individual interval between menopause and first symptoms has a Gaussian distribution with mean at time 0, implying that the average woman develops the first symptoms at the time of her menopause. The F:M ratio of all patients was 2.3; with increasing age the F:M ratio decreased from 3.7 before 30 years of age to 1 after the 6th decade of life, with a peak at the age of 40-44 years. A possible effect of age related changes in sex hormone levels on the pathogenesis of RA is suggested.

Adult

"Microgel diffusion blotting" for sensitive detection of antibodies to extractable nuclear antigens.

A fast immunoblotting procedure, termed "microgel diffusion blotting," is used to detect and identify antibodies to extractable nuclear antigens (i.e., to Sm, RNP, and SSB) in patients with rheumatic diseases. The method differs from the standard immunoblotting techniques by the use of ultra-thin microgels for polyacrylamide gel electrophoresis: the very thin gel layer allows transfer of proteins to a nitrocellulose membrane by simple diffusion. Principal advantages of this variant technique are its simplicity, rapidity, and reproducibility--characteristics that make the test suitable for routine application. We compared the sensitivity of the test with that of double immunodiffusion in agarose for the evaluation of humoral antinuclear immunity. Microgel diffusion blotting detected antibodies in serum at concentrations less than 0.001 of those detectable by immunodiffusion.

Antigens, Nuclear

Microgel immunoblotting of thymus and nuclear extracts by unidirectional diffusion.

A variant immunoblotting procedure is presented, starting from sodium dodecyl sulfate-containing ultra-thin microgels. The use of these gels allows efficient transfer of proteins to an immobilizing matrix such as nitrocellulose, by simple uni-directional diffusion, without loss of resolution. To this end an assembly was developed, keeping the microgel and the immobilizing matrix in continuous contact. To standardize the technique, two protein extracts (a classical rabbit thymus extract and an original autologous nuclear extract) were used, together with reference antinuclear antisera. The method is fast, easy to perform, and perfectly reproducible. For these reasons, the technique is very suitable for screening a patient's sera on a large scale.

Alkaline Phosphatase

Linkage of a polymorphic marker for the type III collagen gene (COL3A1) to atypical autosomal dominant Ehlers-Danlos syndrome type IV in a large Belgian pedigree.

We have examined a large family in which eleven members have a form of autosomal dominant Ehlers-Danlos syndrome type IV. Analysis of fibroblast cultures from affected individuals showed a partial deficiency of type III collagen production. The protein produced was, however, normal in all aspects examined. Using a restriction site polymorphism associated with the structural gene for human type III collagen (COL3A1), we have found tight linkage between the low frequency polymorphic allele and the clinical expression of the disease (lod = 3.86 at 0 = 0), identifying the type III collagen gene as the disease locus.

Adult

Ehlers-Danlos syndrome type I: a clinical and ultrastructural study of a family with reduced amounts of collagen type III.

Ehlers-Danlos syndrome (EDS) type I was diagnosed in an 18-year-old girl on the basis of marked skin hyperextensibility with generalized loose-jointedness, pigmented paper-tissue scars, and a pronounced tendency to bruising. Her father and one of her sisters showed a similar phenotype. Her mother was normal. Light microscopy of skin biopsies showed large, irregular collagen fibres in the father and daughter, with normal findings in the mother. Electron microscopy of the skin sections revealed a variation in diameter and shape of the collagen fibrils as well as slight dilatation of the rough endoplasmic reticulum of fibroblasts in father and daughter, but normal findings in the mother. Cultured fibroblasts did not show these changes. Measurements of collagen synthesis by fibroblast cultures showed that type III collagen levels were reduced to 50% of normal in the father and daughter, and were normal in the mother. The alpha I (III) proteins had a normal molecular weight, determined by SDS-PAGE electrophoresis. The phenotypes and biochemical results in the family members tested were compatible with autosomal dominant transmission. To our knowledge, this is the first report of a type III collagen deficiency in Ehlers-Danlos syndrome type I. The findings in this family, especially the pronounced bruising tendency, illustrate the heterogeneity within type I EDS.

Chromatography, Thin Layer