Gluten, a lectin with oligomannosyl specificity and the causative agent of gluten-sensitive enteropathy.
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Biomedical subjects
Publications and source records attributed to B Volk.
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Alcohol was administered in a vitamin-supplemented liquid diet (Stardit) to Wistar rats 4 weeks before mating and during pregnancy. Of the daily calories 38--40% were supplied by alcohol and by isocaloric sucrose in the controls. Offspring of alcohol drinking dams showed a reduced birth weight and gained weight less rapidly than the controls. On day 3, 4, 7, 12, 17 and 21, experimental animals and controls were perfused for histological and electron microscopic investigations. Morphometric analysis of the cerebella (Fol. 5 and Fol. 8) showed no difference in the number of Purkinje cells. However, on day 7 the Purkinje cell nuclei of experimental pups were significantly smaller. This difference disappeared at day 12. Electron microscopic investigations in the 4, 7, and 12 days old experimental animals revealed a delayed cytoplasmic maturation of Purkinje cells which mainly involved the rough endoplasmic reticulum. At 17 days, there was no difference between the two groups. These data are discussed in relation to alcohol-induced metabolic changes in the brain.
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Maternal chronic ethanol abuse during pregnancy causes malformations of the offspring. Three children (aged 6 months, 9 months, 4 1/2 years) and 3 fetuses (17th, 18th, and 20th gestational week) showed a wide spectrum of disorders ranging from severe dysraphic state, arhinencephaly, porencephaly, agenesis of corpus callosum, a range from hydranencephaly to microdysplasias (p.e. reduced gyration of dentate nucleus and inferior olives), and a range from gastrochisis or congenital heart defects to craniofacial dysmorphogenesis and palmar crease anomalies. The patterns of the cerebral malformations were not as uniform as the clinical phenotype of the alcohol embryopathy. The observations did not support the assumption that there exists a specific period for alcohol teratogenicity.
Considerable intraaxonal lipid storage was observed in intramuscular nerve fibres mainly of the extraocular muscles and less frequently in limb muscles of a 3-year-old child, which had suffered from Tay-Sachs' disease. Many axons of the small intramuscular nerve twigs and of the terminal and preterminal nerve endings showed spherical and cylindrical enlargements containing granular storage material of the same staining properties as the material in the central nervous system. The identify of the axonal and the neuronal storage material was further confirmed by electron-microscopical demonstration of typical multilamellated cytoplasmic bodies (MCBs) in both of them. In addition, silver impregnation and electron microscopy revealed dystrophic changes with an increase of argyrophilic filamentous material within some of the axonal distensions, indicating that the latter were probably due to both a lipid storage and an unspecific dystrophic process.
A case with peripheral neuropathy due to monoclonal gammopathy is reported. There was striking, dissociated symmetrical sensory loss and paresis, the legs being affected predominantly. The case is compared with the few reported cases of myelomatous neuropathy, and the clinical and histological findings are discussed in view of the pathogenesis.
The cerebellar postnatal development of young rats (Wistar) was investigated, whose mothers were given a 12% (W/V) ethyl alcohol solution for 7 days before and during pregnancy. Besides weighing less at birth, the experimental animals had a slower postnatal linear growth and gained weight less rapidly. Histological examination of the cerebellum showed an inpeded histogenesis, which could be observed in the Purkinje cells up to the 10th postnatal day. Moreover, a thick layer of embryonal granular cells with numerous mitoses could still be seen in the experimental animals at 3 weeks of age. After 30 days there was no histological difference between the controls and experimental animals. Because the Purkinje cells in the developing cerebellum represent the units were ribosomes are most abundant, it seems appropriate to ask, whether the observed inhibition of histogenesis could derived from an impaired cerebral protein synthesis. Disturbance of organogenesis was observed in three experimental animals afflicted with an extreme internal hydrocephalus.
A 40-year-old man suffered from a right parietal oligodendroglioma. Two months after the onset of clinical symptoms complete removal of the tumor was attempted. At second craniotomy four years later the tumor was found to invade the falx cerebri. The patient died seven years after the first admission to the hospital. Necropsy revealed the right parietal oligodendroglioma invading the sagittal sinus and the left hemisphere as well as metastases in the lung, the lymph-nodes, the vertebra, and the femur.
In a muscle biopsy from a patient suffering about 2 years from an amyotrophic lateral sclerosis groups of atrophic fibres and target fibres were observed. By electron microscopic examination, amongst numerous parallel or spinal cisternae near the plasma membrane, single-membrane bound smooth tubular elements with a constant diameter of 200-300 A occur in the central and intermediary zone of some target fibres. The connexion between these tubules and the terminal cisternae (triads) of the sarcoplasmic reticulum, suggest that this phenomenon seems to be a proliferation of the transversal-tubular system due to the partial denervation. Only a mechanical displacement of the T-tubules as a result of the destruction of the muscle fibres is not plausible, because the T-system volumetrically amounts only 0.3-0.5% of the muscle fibre.
Both the corpora amylacea and Lafora bodies have been described in the literature as filamentous structures. On electron microscopic examination they are often composed of ribbon-like fasciolar units. The ultrastructural appearance of these structures is discussed on the example of an amylopectin model. These fasciolar substructures are also encountered in Bielschowsky bodies and are then considered to be a sign of intracellular accumulation of dysmetabolic products. The histological and electron microscopical similarity of corpora amylacea, Lafora bodies and Bielschowsky bodies suggest that all these structures are an unspecific, nearly identical endproduct of various intracellular metabolic disturbances. The pathognomonic significance of these structures depends on their regional distribution: the common corpora amylacea occur predominantly in the astroglia, the Lafora bodies typical in the neurons and the Bielschowsky bodies are restricted to the neurons of the exterior pallidum.
On a rat, which has spontaneous died 12 months after 4 times i.p. application of aflatoxin during pregnancy, there has been found a frontal tumor barely of the size of a pepper-corn which came out from the leptomeninx. A comparison to the Abrikossoff tumor concerning the light- and electron-microscope as well as the nucleic-acid concentration is justified. The protein content of the granula of the tumor cells is higher than the values known in the granular-cell myoblastoma of man.
In the m. tibialis anterior of a 68-year-old man with rapidly developing denervation atrophy in the legs since 1/2 year prior to death from heart stroke, abundant unifocal concentric fiber changes, such as target, targetoid/core, and targetoid fibers could be observed. Besides, large vacuolized fibers with multiple changes resembling cytoplasmic bodies in the peripheral zone were present as well; they are interpreted as fibers with multicentric target or targetoid formations. The target fibers displayed a broad variation of their outer appearance suggesting a continuous transition to targetoid/core fibers (with a dense center) and targetoid fibers (with a central change to aquous sarcoplasm showing a paucity of fibrillar structures). Very few fibers with a central densification of fibrillar material with or without a thin intermediate zone were fairly akin to core fibers of central core disease; others were more alike the type of targetoid fibers, previously described in the literature, showing a dense target-like center; both were summarized under the term, inaugurated by Engel et al. (1966), "targetoid/core fibers". Simultaneous occurrence of the different kinds of concentric fiber changes suggested a strong relation between all of them in the sense of representing different developmental stages of the same pathogenetic process. Thus, the central core disease, for instance, might be a disorder with a generalization of concentric fiber changes having come to arrest in the earliest stage of development.
In a 60-year man with a very slowly progressive motor disturbance probably of torsion systonic or choreoathetotic type which started in childhood, we found an isolated degeneration of the exterior pallidum with intraneuronal deposition of Bielschowsky bodies and only slight deficit of neurons. The Bielschowsky bodies show the same structure as corpora amylacea and Lafora bodies. They might also be the result of a cellular disturbance mainly concerning the carbohydrate metabolism. In this case they are localised only in the neurons of the exterior part of the pallidum--in contrast to the organogenitically otherwise deriving (from the nucl. entopeduncularis) intact neurons of the pallidum internum--and they induce, after an excessive accumulation of the metabolites, not till extremely late loss of neurons. This circumstance explains the very torpid progress of this disease.