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Biomedical subjects

E Bonilla

Publications and source records attributed to E Bonilla.

At least 217 records · Page 12Linked to original sources

Freeze fracture studies of muscle plasma membrane in human muscular dystrophy.

Freeze fracture analysis of intramembranous particle density in skeletal muscle plasma dystrophy from 7 patients with Duchenne muscular dystrophy (DMD), 5 patients with facioscapulohumeral muscular dystrophy (FSH) and 5 patients with myotonic dystrophy (MyD) were carried out. Marked depletion of intramembranous particles including orthogonal arrays was noted in DMD while only orthogonal arrays were significantly decreased in FSH. No abnormalities were noted in MyD.

Adolescent↗

Tyrosine hydroxylase activity in Venezuelan equine encephalomyelitis virus infection.

In rats inoculated with the Venezuelan equine encephalomyelitis (VEE) virus, a significant decrease was found in the tyrosine hydroxylase activity of neostriatum, midbrain, and hypothalamus during the acute phase of the infection. In animals that survived the acute infection, we observed no changes in the enzymatic activity in the same regions studied. Our findings suggest a vulnerability of the dopaminergic and noradrenergic pathways to the infection produced by VEE virus.

Animals↗

[Treatment of chromoblastomycosis with amphotericin B and 5-fluorocytosine].

The authors are confirming the efficacy of the combination of Anfotericina B y 5-fluorocitosine in the treatment of Cromoblastomicosis. The used program was as follows: 50 mg. of Anfotericine-B by intravenous injection every two days during 13 weeks (39 dosis). Simultaneously tablets of 500 mg. of 5-fluorocitesina, every 6 hours, oral, also daily during 13 weeks (194 gramos dosis total) were given. The two treated patients were under monthly control during 10 months and observing no relapse of the disease, with other words there exists already a clinical and histological cure (healing). In one case it was observed a change in the ble blood chemistry which spontaneously normalized when suspending the combined treatment for one week and did not repeat when starting a new treatment.

Administration, Oral↗

Freeze-fracture studies of muscle caveolae in human muscular dystrophy.

Freeze-fracture studies of caveolar density and distribution were carried out in skeletal muscle plasma membrane from 6 patients with Duchenne muscular dystrophy (DMD), 5 patients with facioscapulohumeral dystrophy (FSH), 5 patients with myotonic dystrophy (MyD) and 5 normal control subjects. The results showed a significant increase in the number of caveolae and a decrease in size in a population of muscle plasma membranes from patients with DMD. No significant changes in the caveolae were observed in muscle plasma membranes from patients with FSH and MyD.

Adult↗

Water intake and brain choline-acetyltransferase and acetylcholinesterase activites in manganese treated rats.

Choline-acetyltransferase activity was not affected in any of the brain regions studied on the first, third and eighth month of treatment with manganese chloride. Acetylcholinesterase activity diminished in the caudate nucleus only on the eighth month of intoxication but was not altered in any other region throughout the study. From the first month, the manganese-loaded rats ingested an amount of water significantly lower than controls.

Acetylcholinesterase↗

Ultrastructural localization of concanavalin A-binding sites in satellite cells of human skeletal muscle.

Electron-microscopic cytochemical studies on satellite cells of normal human skeletal muscle were carried out using the concanavalin A-peroxidase (Con A-HRP) coupling method. Con A-binding sites, which probably correspond to glycoproteins, were found to be associated with the cell surface, smooth surfaced vesicles, nuclear envelope and endoplasmic reticulum of the satellite cells and were also identified at the cell surface of the adjacent muscle fiber. The possible relationships of these observations to the functions of satellite cells are discussed.

Adult↗

Ultrastructural study of globular inclusions in human skeletal muscle mitochondria.

Limb muscle biopsies from a patient with Luft's disease and a patient with a slowly progressive neuromuscular disorder since infancy revealed by conventional electron microscopy the presence of globular inclusions in the mitochondria as one of the most prominent morphological findings. Electron cytochemical studies on fresh tissue blocks showed no cytochrome c oxidase activity within the globular inclusions. The study of strontium uptake supported by either NAD and flavo-protein linked substrates in freshly isolated mitochondria fractions showed no electron-dense needles within the globular inclusions. Attempts to remove the inclusions with pepsin and with pronase on ultrathin sections failed but they were partially and totally removed by treatment of the sections with hydrogen peroxide. Freeze fracture studies showed the globular inclusions consisted of amorphous and lamellar material. The results suggest that the globular inclusions in muscle mitochondria may consist primarily of lipid.

Freeze Fracturing↗

GABA metabolism in Venezuelan equine encephalomyelitis virus infection.

Mice infected with the Venezuelan equine encephalomyelitis virus showed a significant decrease in the GABA content of cerebral hemispheres. Activity of the enzyme which synthetizes GABA, glutamate decarboxylase, is also reduced in whole cerebral hemispheres, neostriatum, and frontal cortex of infected animals, as compared to values obtained from the same regions of control mice. No significant difference was demonstrated in the activities of GABA transaminase, glutamate dehydrogenase, lactate dehydrogenase, succinate dehydrogenase and NAD-malate dehydrogenase in any of the regions studied. The results suggest that the viral infection produced an alteration in the mechanism of GABA synthesis.

Animals↗

Transplantation of human skeletal muscle to nude mice: a sequential morphologic study.

Normal human quadriceps muscle was transplanted into nude mice subcutaneously for periods of 1, 2, 3, 4, 6, and 8 weeks. At 1 week, there was myoblast and myotube formation with thin and thick filaments. After 2 weeks, there was myotube formation with partially cross-striated myofilaments, and formation of dyads or triads. Leptomeres were also noted in some myotubes. By 4 weeks, cross-striation of myofilaments and migration of muscle nuclei to the periphery were observed. Distended sarcoplasmic reticulum and honeycomb structures of tubules were noted between 4 and 8 weeks. This study indicates that mature human muscle can be transplanted successfully into nude mice, providing another tool for the investigation of human neuromuscular disease.

Animals↗

L-tyrosine hydroxylase activity in the rat brain after chronic oral administration of manganese chloride.

In rats chronically treated with a high oral load of MnCl2 a significant increase in the activity of L-tyrosine hydroxylase was observed in neostriatum, midbrain and hippocampus one month after the beginning of the experiment. The augmented enzymatic activity persisted in neostriatum, midbrain and hypothalamus on the third month and remained elevated only in neostriatum on the sixth month. After eight months a significant decrease in the activity of the enzyme was found in neostriatum with no changes in the remaining regions studied. These findings are interesting since human manganese intoxication starts with a psychiatric phase bearing similarities to schizophrenia in which the primary disturbance has been suggested to be an overactivity of dopamine neurons. On the contrary, the permanent neurological phase is associated with reduced striatal dopamine, presumably due to a decrease in L-tyrosine hydroxylase activity.

Administration, Oral↗

Quantitative ultrastructural study of muscle satellite cells in Duchenne dystrophy.

Quantitative ultrastructural studies of satellite cell populations, and nuclear chromatin analysis of satellite cell nuclei and true subsarcolemmal nuclei were carried out in normal and Duchenne muscular dystrophy (DMD) muscles. There was a remarkable increase in the number of satellite cells in muscles of patients with mild to moderate stages of DMD, as compared to preclinical or very early and probably advanced stages of DMD, and a significant increase in euchromatin content was found in the satellite cell nuclei in DMD. Since it is generally accepted that satellite cells participate actively in muscle fiber regeneration, this study suggests that muscle fiber regeneration is ineffective in DMD.

Adolescent↗

Freeze-fracture studies of erythrocyte plasma membrane in human neuromuscular diseases.

Freeze-fracture studies were conducted in erythrocyte plasma membrane from 8 patients with Duchenne muscular dystrophy (DMD), 8 age-matched controls, 3 adult controls, 10 patients with myotonic muscular dystrophy, and 26 other neuromuscular disease controls. There was marked depletion of intramembranous particles in Duchenne dystrophy, whereas intramembranous particle density counts in other neuromuscular diseases were within normal limits. Therefore, the internal molecular architecture of the erythrocyte membrane is abnormal in Duchenne dystrophy, supporting the concept that a membrane defect involving multiple tissues is present in this disorder.

Erythrocyte Membrane↗

Lamellar bodies at the cell periphery of human muscle fibers.

The cytochemical and ultrastructural features of lamellar bodies in human skeletal muscle fibers were studied using tannic acid-glutaraldehyde, ruthenium red-glutaraldehyde fixation methods, conventional electron microscopy and the freeze fracture technique. The lamellar bodies consisted of concentric lamellae with a regular spacing of 6.5 +/- 0.2 nm. These structures were found preferentially at the cell periphery closely associated with the plasma membrane, near the nuclear poles and in the space between muscle fiber and satellite cell. The cytochemical and ultrastructural features of the lamellar bodies suggest they are largely composed of phospholipid. It is possible that these structures are involved in muscle membrane maintenance.

Adult↗