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  • 1
    ISSN: 1520-510X
    Source: ACS Legacy Archives
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Cellular and molecular life sciences 42 (1986), S. 155-157 
    ISSN: 1420-9071
    Keywords: Protease ; amidase ; macrophage ; guinea pig
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Proteolytic enzymes of the guinea pig peritoneal exudate macrophages were investigated using synthetic fluorogenic peptide substrates. Among several enzymes, t-butyloxycarbonyl-phenylalanyl-seryl-arginine 4-methylcoumaryl-7-amide cleaving enzymes had the highest activity, and the activity in exudate macrophages was about 3 times tronger than that in resident thiol-blocking reagents, suggesting a thiol protease.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0533
    Keywords: Key words Neuronal intranuclear inclusion ; Neurodegenerative diseases ; Polyglutamine ; Ubiquitin ; Immunohistochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Neuronal intranuclear hyaline inclusion disease (NIHID) is a group of neurodegenerative disorders characterized by the presence of intranuclear inclusions in neurons (NIs). We report here clinicopathological findings of a 25-year-old female patient who died after 13 years of a clinical course characterized by progressive gait disturbance and movement disorders. Histological examination revealed widespread NIs with neuronal loss in restricted regions; neuronal loss was severe in the subthalamic nucleus, internal pallidum, substantia nigra, Edinger-Westphal nucleus and Purkinje cell layer. Quantification of the NIs combined with a graded evaluation of neuronal loss revealed an overall tendency for more severe neuronal loss to be accompanied by a lower frequency of NIs. A morphological similarity to the nuclear inclusions recently identified in several CAG repeat diseases prompted us to examine the immunolocalization of ubiquitin and expanded polyglutamine stretches, which demonstrated the presence of ubiquitin at the periphery of most NIs. An expanded polyglutamine stretch was seen in the center of limited number of NIs. These findings indicate that abnormal fragments such as expanded polyglutamine regions are incorporated into the inclusion, aggregated in its center, and thereby metabolized by a ubiquitin-dependent proteolytic pathway. Although it remains to be elucidated how the formation of NIs is related to neuronal degeneration, our findings suggest that NIs are formed in the process of sequestering or degrading abnormal protein fragments and formation of NIs may not be immediately toxic to neurons.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-0533
    Keywords: Mitochondria ; Parkinson disease ; Substantia nigra ; 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) ; Monkey
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Nigral neurons of crab-eating monkeys treated with 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) showed a peculiar configuration occasionally in mitochondria. The outer membrane of mitochondria was covered with a net of fine parallel or latticed filaments, which turned spirally about the long axis of the mitochondria. The filaments were approximately 8 nm in diameter: parallel filaments were arranged at intervals of about 13 nm from center to center; and latticed filaments intersected each other at an angle of almost 135°. When mitochondria were present in groups, the intermitochondrial gap was occasionally filled with the same parallel filaments. The netted mitochondria were frequently associated with intramitochondrial abnormalities such as small flocculent inclusions and disintegrated cristae. Only one or two netted mitochondria were counted in the perikaryon of one section of an injured neuron. They appeared in about one-third of mildly or moderately injured neurons in three of six MPTP-treated monkeys, and not in normal surviving and recovering neurons of treated animals, or in neurons of control animals. We consider the netted mitochondria to be a pathological configuration related to a metabolite of oxidation of MPTP, and to be different from the stubby mitochondria reported in amyotrophic lateral sclerosis (ALS) and a non-ALS case.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-0533
    Keywords: Striatonigral degeneration ; Putaminal pigment ; Iron ; Atomic absorption spectroscopy ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We measured the total content of iron, cooper, zinc and manganese in the putamen of four patients with striatonigral degeneration (SND) and age-and gender-matched normal controls. The iron content in the SND patients was five times greater than in the controls. Electron microscopic histochemistry revealed the iron reaction products in the pigments showing a triphasic pattern of coarse, electron-dense globules, fine granular and fibrillary materials, and lamellated structures. These findings suggest that increased iron deposition may be related to pigment formation in the putament of SND.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-0533
    Keywords: Alzheimer's disease ; Cerebral cortex abnormalities ; Muscular dystrophy, congenital ; Neurofibrillary tangles ; Paired helical filaments
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Three patients with Fukuyama congenital muscular dystrophy (FCMD), who died aged 23, 29 and 34 years, are reported. There was extensive brain malformation, but the most severe pattern of cortical dysplasia was absent in one case and in the other two localized bilaterally to small areas near the occipital poles. In two cases, numerous neurofibrillary tangles were observed in the locus ceruleus and nucleus basalis of Meynert. Electron microscopy revealed paired helical filaments, with a maximal width of about 25 nm and regular constrictions at approximately 80 nm intervals. The occurrence of neurofibrillary tangles, which is unknown in younger patients, suggests the presence of degenerative processes in the brains of the older patients with FCMD.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 64 (1984), S. 22-29 
    ISSN: 1432-0533
    Keywords: Famihal ALS ; Oculomotor impairment ; Urinary disturbance ; Multisystemic degeneration
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Two sibling cases of familial amyotrophic lateral sclerosis (ALS) revealed degerneration usually associated with other systemic degenerative disorders. The changes in the 41-year-old sister were compatible with those reported in other familial ALS cases affecting the upper and lower motor neurons, posterior columns, and spinocerebellar tracts. The 45-year-old sister revealed more wide-spread degenerative changes involving not only motor neuron systems, but also proprioceptive, general somatic afferent and spinocerebellar afferent systems. Intracytoplasmic hyaline inclusions were observed in the oculomotor nuclei. Clinical manifestations of urinary disturbance and oculomotor imparirment seldom seen in sporadic ALS were interpreted to be due to the unusual distribution of the morbid process. These pathologic findings suggest that familial ALS may be a multisystemic degenerative disorder, frequently involving the spinocerebellar tracts, but occasionally involving other systems as well.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-0533
    Keywords: 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-induced parkinsonism ; Crab-eating monkey ; Substantia nigra ; Locus ceruleus ; Mitochondrial abnormalities
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Experimental parkinsonism was induced by systemic administration of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) to the crab-eating monkey (Macaca fascicularis). In the acute stage, the substantia nigra showed necrotic nerve cells, extracellular release of pigment granules and histiocytic infiltration. The nerve cells underwent vacuolation of the cytoplasm with chromatin clumping of the nucleus and disintegration of the nucleolus. The striking feature was the presence of variously-shaped inclusion bodies within abnormal mitochondria which sometimes disclosed a distortion of the cristae. Golgi apparatus and endoplasmic reticulum were also dilated. In the locus ceruleus swollen nerve cells were observed with vacuolated cytoplasm and pyknotic nucleus where expanded mitochondria also contained the inclusions. Some of the inclusion bodies are probably insoluble precipitations due to inhibition of mitochondrial oxidation by a certain metabolite of MPTP. In the protracted stage the substantia nigra revealed a considerable loss of the nerve cells associated with melanophagia and astrocytic proliferation. A few surviving nerve cells showed an increased number of Golgi apparatus and rough endoplasmic reticula, and the presence of autophagosomes, dense bodies and intra-mitochondrial inclusions. These changes are interpreted as being a part of the reparative process from the cellular damage.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-1793
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology
    Notes: Abstract Cell growth of a coastal marine diatom, Chaetoceros sociale, in the presence of different premixed organic-Fe(III) complexes [EDTA-Fe(III) (100:1 and 2:1), citric-Fe(III) (100:1) and fulvic-Fe(III) (0.1, 0.2 and 1 ppm C)] and solid amorphous hydrous ferric oxide [am-Fe(III) or Fe(III) hydroxide] were experimentally measured in culture experiments at 10 °C under 3000 lux fluorescent light. Fulvic-Fe(III) (0.1 and 0.2 ppm C) and citric-Fe(III) (100:1) induced maximal cell yields of C. sociale. The order of cell yields was: fulvic-Fe(III) (0.1 and 0.2 ppm C) ≥ citric-Fe(III) (100:1) 〉 EDTA-Fe(III) (2:1) ≫ solid am-Fe(III) 〉 EDTA-Fe(III) (100:1) ≫ fulvic-Fe(III) (1 ppm C). The short-term iron uptake rates by C. sociale in fulvic-Fe(III) (0.1 and 0.2 ppm C) and citric-Fe(III) (100:1) media were about five to six times faster than those in EDTA-Fe(III) (100:1) and solid am-Fe(III) media. The dissociative precipitation rates of premixed organic-Fe(III) complexes in seawater at 10 °C were determined by simple filtration (0.025 μm) involving γ-activity measurements of 59Fe. The order of estimated initial Fe(III) dissociative precipitation rates of these organic-Fe(III) complexes in seawater were nearly consistent with those of cell yields in the culture experiments and short-term iron uptake rates by C. sociale [except for fulvic-Fe(III) (1 ppm C) medium]. In fulvic-Fe(III) (0.1 and 0.2 ppm C), citric-Fe(III) (100:1) and EDTA-Fe(III) (2:1) media, the concentrations of dissolved organic-Fe(III) complexes in initial culture experiments are prone to supersaturate under the culture conditions. The supersaturated dissolved organic-Fe(III) complex in seawater supplies biologically available inorganic Fe(III) species in culture media through its dissociation at high pH and high levels of seawater cations. Therefore, the natural dissolved organic-Fe(III) complexes supplied by riverine input may play an important role in supplying bioavailable iron in estuarine mixing system and coastal waters.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-1106
    Keywords: A1 noradrenergic region ; Subfornical organ ; Paraventricular neurohypophyseal neuron ; Microiontophoresis ; Adrenoceptor antagonist
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The action of the A1 noradrenergic neurons of the ventrolateral medulla on the responsiveness of neurohypophyseal neurons in the rat hypothalamic paraventricular nucleus (PVN) to inputs from the subfornical organ (SFO) was examined in antidromically identified PVN neurons that respond to electrical stimulation of both the SFO and A1 region. In both putative vasopressin (VP)- and oxytocin (OXY)-secreting PVN neurons that were classified according to their spontaneous firing patterns and their responsivity to baroreceptor activation, prior stimulation of the A1 region did not affect the short latency brief duration excitatory response induced by SFO stimulation. Simultaneous stimulation of the A1 region significantly enhanced the long latency prolonged excitatory response induced by SFO stimulation and the enhancement was blocked by microiontophoretically applied phentolamine, and α-adrenoceptor antagonist, but not by timolol, a β-adrenoceptor antagonist. Simultaneous stimulation of the A1 region also significantly enhanced the inhibitory response induced by SFO stimulation and the enhancement was blocked by microiontophoretically applied timolol, but not by phentolamine. These results suggest that the A1 region may act to enhance the partial excitatory (via an α-adrenoceptor mechanism) and inhibitory SFO inputs (via a β-adrenoceptor mechanism) to the PVN neurohypophyseal neurons as a modulatory action.
    Type of Medium: Electronic Resource
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