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Biomedical subjects

H Bernheimer

Publications and source records attributed to H Bernheimer.

78 records · Page 5Linked to original sources
PubMedDate not supplied

[Lipid storage diseases (lipidoses): genetic, biochemical and clinico-chemical aspects].

Lipidoses are genetic diseases due to disease-specific defects in the enzymatic catabolism of lipids, with accumulation of the respective lipid substrate in the nervous system and/or peripheral tissues. The clinical chemical diagnosis of lipidoses can be accomplished by demonstration of the enzyme defect and/or substrate accumulation in body fluids (urine, blood serum), leukocytes, cultured fibroblasts, amniotic fluid cells, or amniotic fluid, respectively. These assays are important with regard to: 1. the specific detection or exclusion of diseases, which are difficult to diagnose by their clinical presentation, 2. prenatal diagnoses, 3. detection of (clinically inconspicuous) heterozygotes (essential for individual genetic counselling), and 4. the biochemical control of dietary treatment in Refsum's disease.

Brain Diseases, Metabolic↗
PubMedDate not supplied

Antisera against ganglioside GM2: immunochemical and immunohistological studies.

Antisera against ganglioside GM2 were raised in rabbits and tested by immunodiffusion, complement fixation and immunohistology. The presence of precipitating antibodies was demonstrated by immunodiffusion techniques (Ouchterlony and reversed Mancini). GM2-antibody titres were determined by a quantitative microcomplement fixation assay. The GM2-antibodies were shown to be specifically directed against GM2 and did not cross-react with the major brain gangliosides. Application of GM2-antiserum to brain sections from a case of GM2-gangliosidosis (Sandhoff's disease) in an immunofluorescence study produced a specific fluorescence of the granular GM2-storage material within the cytoplasm of cortical neurons. With immunoperoxidase staining by electron microscopy, labelling of the surface of the membraneous cytoplasmic bodies was obtained.

Brain↗
PubMedDate not supplied

Dynamics of IgG+, IgA+, and IgM+ plasma cells in the central nervous system of guinea pigs with chronic relapsing experimental allergic encephalomyelitis.

IgG+, IgA+, and IgM+ plasma cells (PC) were investigated by quantitative immunocytochemistry in the central nervous system (CNS) of guinea pigs with chronic relapsing experimental allergic encephalomyelitis (crEAE) and in the CNS of controls. Frequencies of Ig+ PC in crEAE were, on average, 30-130 times higher than in controls. At all stages of crEAE, IgG was the dominant isotype of CNS PC. In the course of crEAE there was an increase in the proportion of IgG+ PC and a decrease in the proportions of IgM+ and, to a lesser extent, of IgA+ PC. Accumulation of IgM+ PC appears to be related to the acuteness of the disease. Our studies also indicate that IgA, in addition to IgG and IgM, is involved in the immunological response of crEAE in the CNS, and demonstrate that the time course of crEAE is characterized by isotype-specific differences in the immune responses of IgG+, IgM+, and IgA+ PC in the CNS.

Animals↗