Altered distribution of lamin and emerin in muscle nuclei of sIBM patients.

OBJECTIVE: Sporadic inclusion body myositis (sIBM) is a chronic acquired inflammatory myopathy. The cause of sIBM remains unknown and its pathogenesis is controversial. There is a hypothesis [Karpati and Carpenter 1993] that the rimmed vacuoles result from nuclear breakdown, and IBM filaments are formed from components of the nuclear matrix. MATERIAL AND METHODS: For nuclear membrane protein detection, six IBM patients were studied using immunohistochemical and immunochemical techniques. RESULTS: It was demonstrated that in the interior of 10-15% myonuclei emerin and lamin A/C inclusions appeared constantly. This finding indicated an abberant localization of lamin A/C epitopes, the presence of presumptive lamin A (67 KDu) and emerin as in the affected nuclei. CONCLUSION: We support the suggestion that truncated, changed lamin A protein takes part in nuclear disintegration and rimmed vacuole formation.

Ultrastructure of peripheral blood lymphocytes in some degenerative central nervous system disease.

The ultrastructure of peripheral blood lymphocytes of 30 children with degenerative central nervous system diseases was analyzed. The affected children were divided into four groups. Lysosomal storage were characterized by the storage of membrane-bound inclusions in peripheral lymphocytes. Ceroidlipofuscinosis was manifested by the presence of curvilinear bodies. The appearance of abnormal mitochondria was found in mitochondrial encephalopathies. The tubuloreticular structures seen in lymphocytes of some unclassified cases suggested viral factors that acted in prenatal life. All findings confirm the role of peripheral lymphocytes analysis in the diagnosis of degenerative central nervous system diseases.

Inclusion body myositis (IBM). Morphological study.

Among the chronic idiopathic inflammatory myopathies inclusion body myositis (IBM) has emerged as a clinicopathologic variant. Slowly progressive weakness of the distal and the proximal muscle groups, the presence of rimmed vacuoles with basophilic granules as well as 15-18-nm filamentous inclusions in affected muscle confirm the clinical and histopathological distinction between inclusion body myositis and chronic polymyositis.

Type III collagen deficient EDS IV producing muscular hypotonia with abnormal muscle fibroblasts.

Muscle biopsy and fibroblast culture of a floppy child with Ehlers-Danlos syndrome type IV were studied. Biochemical analysis of the tissue showed drastically reduced amount of collagen type III. Electron microscopic examination of muscle as well as of cultured fibroblasts showed grossly dilated and dominated the cytoplasm endoplasmic reticulum. Dilatation may result from storage of an abnormal collagen type III molecule. Our observations indicate that type III collagen deficiency may be present clinically as a congenital muscle hypotonia. Specific ultrastructural abnormalities of fibroblasts found in muscle biopsy can enable the proper diagnosis.

Muscle cell death. Ultrastructural differences between muscle cell necrosis and apoptosis.

The morphology and incidence of muscle cell necrosis and apoptosis are presented. Necrosis which occurs as a massive tissue damage is structurally characterized by swelling of the muscle cell and disruption of cellular components. Apoptosis, on the other hand, is a process of active cellular self-destruction. It shows characteristic sequence of muscle cell shrinkage, which is ending in transformation of each muscle cell into compact apoptotic bodies that in turn are phagocytized by adjacent cells or macrophages.

An ultrastructural study of the vascular and muscular basement membrane in Duchenne-type dystrophy.

The ultrastructural distribution of basement membrane around the capillaries as well as muscle cells of boys with Duchenne-type muscular dystrophy was determined. In dystrophic muscles, a diffuse thickness of vascular as well as muscular basement membrane was observed. Lamina densa lost its regular ribbon-like appearance and was split into several thin layers. After staining with tannic acid (TA), a densely stained meshwork was present on the muscle cell surface as well as in the extra-cellular space. Hyaluronidase treatment removed TA-stained deposits, indicating that hyaluronic acid is a major component. Enzyme resistant structures, presumably fibrous long-spacing collagen fibrils, have been found in dystrophic muscles. Based on the results of the study, faulty structure of the basement membrane in dystrophic muscle is suggested.

A Kinetic Analysis of Phytochrome Controlled Mesocotyl Growth in Zea mays Seedlings.

Mesocotyl elongation in 4 day old etiolated seedlings immediately following 3 hours of white light (3 h W) is reversibly controlled by phytochrome. Time-lapse video measurements were made of the 5 millimeter zone just below the coleoptile which is the main growth region of the mesocotyl. The growth kinetics were determined for five contiguous 1 millimeter zones subtending the coleoptile node for nonirradiated seedlings, for seedlings given 3 h W, and 3 h W followed by terminal far-red (FR) or red subsequent to the far-red (FR/R) irradiation. Each zone in nonirradiated seedlings exhibits exponential elongation kinetics during the early stages of elongation. This finding suggests that during elongation, a growth limiting factor is also exponentially increasing. Following 3 h W differences in the kinetic responses were found for each zone. In all zones, the inhibitory effect following the 3 h W is totally FR reversible. The effect of FR is reversed by R. The upper zone exhibits the fastest response and is the most plastic in its growth response. The three upper zones all exhibit spontaneous and sharp recoveries with time. It is suggested that the control by phytochrome is not inductive but rather continuous, the controlling factor being either the level of the far red-absorbing form of phytochrome (Pfr) or the ratio Pfr to total phytochrome.

Ultrastructural study of motoneurons in Werdnig-Hoffmann disease.

Ultrastructural studies of the lumbar spinal cord in three children with Werdnig-Hoffmann (W-H) disease type Ia revealed numerous small neurons which appeared both atrophic and immature. We compared these motoneurons with anterior horn cells of a 3-month-old child, a 27-week and a 16-week human fetus, and found (1) that the motoneurons were much smaller in W-H disease, and (2) the Nissl substance was peripherally located and less developed. Signs of motoneuron immaturity as well as secondary degenerative changes suggest that in W-H disease neurons die either because they fail to make adequate peripheral contact or because the neurons are genetically intrinsically defective.

Abscisic Acid promotes both volume flow and ion release to the xylem in sunflower roots.

The effect of abscisic acid (ABA) on the exudation process in excised sunflower root was investigated. A promotion of both ion flux and volume exudation rate was observed. Cutting off the external supply of ions to the roots markedly increased the magnitude of the ABA effect. The promotive effect of ABA on exudation rate was extremely rapid (less than 6 minutes) and seemed to be biphasic. Reasons are given for relating the ABA effect to two separate actions, namely: (a) on water permeability; and (b) on the release of ions both from vacuoles to the cytoplasm and from symplasm to the xylem. Kinetin inhibited movement of ions to the xylem and its effect did not appear to be related to that of the ABA.

[Benign infantile epilepsy]. / Lagodna padaczka dziecieca.

The authors describe clinical and electroencephalographic analysis of 23 cases of banign infantile epilepsy. The characteristic features of seizures, the clinical course, and the EEG findings with presence of spikes and/or spikes and slow waves in leads from the motor and midtemporal areas are discussed. Frequent influence of factors releasing the first and subsequent seizures and a history of risk factors during pregnancy, and perinatal period, and early acquired predisposition were demonstrated. Follow-up showed that seizures disappeared at the age of puberty. All observations suggest the possibility of separation of benign infantile epilepsy as a particular form of this disease.

Effects of Abscisic Acid and of Hydrostatic Pressure Gradient on Water Movement through Excised Sunflower Roots.

The effect of abscisic acid on the exudation rate from decapitated roots of sunflower plants (Helianthus annuus L.) was investigated in the presence and absence of an imposed hydrostatic pressure gradient. The magnitude of the abscisic acid effect was constant even when suctions up to 60 cm Hg were applied to the cut stumps.When roots were bathed in a THO-labeled nutrient solution, the course of the appearance of radioactivity in the exudate, expressed as a function of exudate volume, was not affected by abscisic acid treatment but was strongly speeded up by applying suction.The implications of those findings with regard to the water pathway through the root and the location of the abscisic acid effect are discussed.

[Problem of epilepsy prevention in children with abnormal EEG tracings following cranial trauma]. / Problem zapobiegania padaczce u dzieci z nieprawidlowym zapisem EEG po przebytym urazie czaszki

In the analysed material of 248 children with past craniocerebral trauma epilepsy developed in 25 cases (10%). In the group of post-traumatic epilepsy seizures developed twice as frequently after severe trauma than after light trauma. In EEG investigations in the group with light trauma the observed changes included slowing down of background activity, focal changes or lateralization as the only finding. In the group of severe trauma the EEG changes included slowing down of background activity with associated lateralization or focal changes. In the group of children with epilepsy following severe trauma the same changes were found in EEG curves prior to development of seizures. The authors believe that changes of this type may predict manifestation of epilepsy and may be regarded as sufficient for prophylactic treatment with anticonvulsants.

Fluxes of a nonelectrolyte and compartmentation in cells of carrot root tissue.

Permeation of thiourea into cells of carrot (Daucus carota L.) storage root discs was found to be governed by its concentration gradient only. The analysis of the course of thiourea efflux from preloaded discs shows two distinct exponential curves, apart from an initial very fast component. Reasons are given for relating the first exponential part of the curve to efflux from the cytoplasm and the second exponential part to efflux from the vacuole.The rate constant for thiourea efflux through the tonoplast is markedly temperature-dependent (activation energy 13.2 kcal mole(-)) and is lowered by 2,4-dinitrophenol and by phenyl mercuric acetate treatments. The rate constant for its efflux through the plasmalemma, on the other hand, has a low activation energy (4.2 kcal mole(-)), which is equal to that of free diffusion of thiourea in aqueous solution, and is not affected by the metabolic inhibitors. Possible reasons for the difference in the properties of the two membranes are discussed.