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  • 1
    Electronic Resource
    Electronic Resource
    FORSSMAN HAPTEN N-ACETYL-α-D-GALACTOSAMINIDASE IN RAT BRAIN AND KIDNEY (1974)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 23 (1974), S. 0 
    Details
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: —Forssman hapten (N-acetyl-α-galactosaminosyl-N-acetyl-β-galactosaminosyl-α-galactosyl-β-galactosyl-glucosylceramide), prepared from sheep erythrocytes was specifically labelled with tritium at the terminal N-acetyl-α-galactosamine moiety by the galactose oxidase-sodium [3H]borohydride method. Activities to cleave the terminal N-acetyl-α-galactosamine from Forssman hapten were detected in the high-speed supernatant of the frozen-thawed and sonicated crude mitochondrial fraction from adult rat brain and kidney. The optimal pH of the reaction was approximately 4·4. The reaction was linear for at least 1 h for the kidney enzyme and up to 3 h for the brain enzyme. Taurocholate was required for the activity. The optimal concentration was 1·5-2 mg/ml. Several other detergents and bile salts tested could not replace taurocholate. The apparent Km of the brain and kidney enzymes were 1·0×10−4M and 3·5×10−4m, respectively. During development, Forssman hapten-cleaving activities of both brain and kidney gradually declined in specific activity as the animal matured. These changes were similar to those of nonspecific p-nitrophenyl N-acetyl-α-galactosaminidase. Several rat organs examined all showed detectable activities to cleave Forssman hapten.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1471-4159.1974.tb04406.x
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  • 2
    Electronic Resource
    Electronic Resource
    GALACTOSYLSPHINGOSINE GALACTOSYL HYDROLASE IN RAT BRAIN: PROBABLE IDENTITY WITH GALACTOSYLCERAMIDE GALACTOSYL HYDROLASE (1974)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 22 (1974), S. 0 
    Details
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract— Activities of rat brain galactosylsphingosine (psychosine) and galactosylceramide (galactocerebroside) galactosyl hydrolases were compared using several criteria. Aqueous homogenates of rat brain were extracted at -30°C with a mixture of ether-methanol (3:1, v/v). This procedure eliminated most of endogenous galactosylceramide and improved the linearity of the enzymatic reaction without inactivating the enzyme. The thermostability of both enzymes was identical while the reference 4-methylumbelliferyl β-galactosidase was less thermostable. The enzymes, solubilized from the ether-methanol powder, were quantitatively precipitated in the combined ammonium sulphate fractions of 20–30% and 30–40% saturation. DEAE-cellulose column chromatography gave identical elution patterns for the two enzymes, with a single major and two minor peaks. Electrofocusing of the major activity peak, obtained from the DEAE-cellulose column, produced a sharp single peak of galactosylsphingosine- and galactosylceramidehydrolysing activities at an isoelectric point of pH 4.45. Developmental changes of these enzymes were identical, showing the most rapid rise concomitant with the period of active myelination. During development, at different purification steps, and in different organs, the ratio of the activities of galactosylsphingosine and galactosylceramide galactosyl hydrolases was relatively constant. While none of these criteria provides definitive proof of identity, they collectively suggest strongly that a single enzyme might catalyse hydrolysis of both galactosylsphingosine and galactosylceramide.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1471-4159.1974.tb11584.x
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  • 3
    Electronic Resource
    Electronic Resource
    SPHINGOGLYCOLIPIDS IN THE NERVOUS SYSTEM IN FABRY'S DISEASE (1972)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 19 (1972), S. 0 
    Details
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract— Two glycolipids, accumulated in the spinal ganglia of a patient with Fabry's disease were identified as: galactosyl (α1 → 4) galactosyl (β1 → 4) glucosyl(1 → 1) ceramide (CTH) and galactosyl (α1 → 4) galactosyl(1 → 1) ceramide (CDG). Only one glycolipid which had the same structure as the CTH in the spinal ganglia accumulated in the sympathetic ganglia of the patient. In the nervous system, CTH contained behenic acid (C22:0) as the major fatty acid. In the spinal ganglia, CDG also contained behenic acid as the major fatty acid.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1471-4159.1972.tb01479.x
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  • 4
    Electronic Resource
    Electronic Resource
    T c increased to 90 K in YBa 2 Cu 4 0 8 by Ca doping (1989)
    [s.l.] : Nature Publishing Group
    Nature 341 (1989), S. 41-42 
    Details
    ISSN: 1476-4687
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Notes: [Auszug] We synthesized samples of Y1_xCaxBa2Cu4O8 (x = 0-0.1) using a high-oxygen-pressure technique. After mixing starting materials of Y2O3, Ba(NO3)2, CuO and CaCO3, the samples were fired at 900 °C in flowing oxygen for 12 h, and then ground. The resulting powder was compacted into a rectangular ...
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1038/341041a0
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  • 5
    Electronic Resource
    Electronic Resource
    Morphometry of the degenerative process in the hypoglossal nerves in amyotrophic lateral sclerosis (1987)
    Springer
    Acta neuropathologica 73 (1987), S. 25-31 
    Details
    ISSN: 1432-0533
    Keywords: Amyotrophic lateral sclerosis ; Hypoglossal nerve ; Morphometrical analysis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The peripheral hypoglossal nerves in 13 cases of amyotrophic lateral sclerosis (ALS) and five control cases were examined using morphometrical methods to demonstrate the degenerative process of motor nerve degeneration. The total number of myelinated fibers and their histograms were analyzed according to the degree of severity of the degeneration. Reduction of the total number of myelinated fibers in ALS hypoglossal nerves were graded in three groups: mild 65%–75%, moderate 50%–65% and severe 30%–50% of the myelinated fibers in controls. Each histogram of the remaining myelinated fibers showed different patterns corresponding to the degree of the degeneration and disclosed that the progressive reduction of large myelinated fibers was the fundamental change. Small myelinated fibers were not reduced, but increased, especially in the group with a moderate grade of degeneration. In plastic section, there were clusters of regenerated myelinated fibers. The transient increase of small myelinated fibers may be a reflexion of myelinated fiber regeneration during the progressive degenerative process of the motor neurons. The correlation between the degree of severity of the hypoglossal nerve degeneration and the atrophy of the tongue muscle and the duration of bulbar symptoms was examined and discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00695498
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  • 6
    Electronic Resource
    Electronic Resource
    An autopsy case of a syndrome with muscular atrophy, decreased subcutaneous fat, skin eruption and hyper γ-globulinemia: peculiar vascular changes and muscle fiber degeneration (1987)
    Springer
    Acta neuropathologica 73 (1987), S. 313-319 
    Details
    ISSN: 1432-0533
    Keywords: Multifocal muscular fibrosis ; Obstructured vessels ; Ischemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary This is the first autopsy case report of a syndrome with autosomal recessive inheritance, muscular atrophy, contracture, skin eruption, hyper γ-globulinemia, decreased subcutaneous fat, mental retardation and abnormal ECG findings. Skeletal muscles showed severe, discrete, multifocal muscular fibrosis which replaced several primary fasciculi. The tongue, heart and extraocular muscles showed identical but less severe findings. In the involved muscle fasciculi, veins and venules as well as arteries and arterioles showed medial hyperplasia and luminal constriction. Degeneration of endothelial cells of arterioles and narrowing of the lumen of terminal arterioles by the debris were observed. The peripheral nerves in the muscles were relatively well preserved. The correlation and pathogenesis of these findings are discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00688252
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  • 7
    Electronic Resource
    Electronic Resource
    Mitochondrial angiopathy in cerebral blood vessels of mitochondrial eneephalomyopathy (1987)
    Springer
    Acta neuropathologica 74 (1987), S. 226-233 
    Details
    ISSN: 1432-0533
    Keywords: Mitochondrial encephalomyopathy (MELAS) ; Mitochondrial angiopathy ; Smooth muscle and endothelial cells ; Pial arteriole and small artery
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We studied cerebral blood vessels of two autopsied patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). All the main cerebral arteries in the proximal portion at the brain base and more distal portion at the cortical surface, as well as within the brain parenchyma were examined by electron microscopy. There was a striking increase in number of mitochondria in the smooth muscle and endothelial cells, which were most prominent in the pial arterioles and small arteries up to 250 μm in diameter and less frequent and severe in the larger pial arteries and intracerebral arterioles and small arteries. These vascular changes have not hitherto been described in MELAS, or in other disorders affecting blood vessels of the brain and other organs. It is suggested that the vascular changes are caused by primary mitochondrial dysfunction in the vascular smooth muscle and endothelial cells of the brain and that they constitute the pathogenic base of the brain lesions and their unusual distribution pattern in MELAS.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00688185
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  • 8
    Electronic Resource
    Electronic Resource
    Adrenomyeloneuropathy: report of a family and electron microscopical findings in peripheral nerve (1985)
    Springer
    Journal of neurology 232 (1985), S. 73-78 
    Details
    ISSN: 1432-1459
    Keywords: Adrenomyeloneuropathy ; Female carrier ; Sural nerve ; Endoneurial cell ; Cytoplasmic lamellar inclusion
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A family with adrenomyeloneuropathy (AMN) is reported, whose female carriers showed severe neurological and biochemical abnormalities. Cytoplasmic lamellar inclusions were found in endoneurial cells of the biopsied sural nerve of a male patient. In spite of the peripheral nervous system involvement in patients with AMN, there have been few reports of these inclusions in the peripheral nerves of such cases.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00313904
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  • 9
    Electronic Resource
    Electronic Resource
    Polymorphic sparteine metabolism and amyotrophic lateral sclerosis (1989)
    Springer
    Journal of neurology 236 (1989), S. 452-455 
    Details
    ISSN: 1432-1459
    Keywords: Amyotrophic lateral sclerosis ; Cytochrome P450 enzymes ; Sparteine sulphate ; Nitro-oxidation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The activity of detoxication with the cytochrome P450 family of enzymes was studied in patients with amyotrophic lateral sclerosis (ALS). The metabolic ratio (MR) of sparteine sulphate was measured, and the population of phenotypes for sparteine nitro (N)-oxidation was determined in 30 patients with ALS and 41 controls for the assay of the sparteine N-oxidation by the cytochrome P450 enzymes. A lower MR and higher frequency of efficient metabolizers were observed in the ALS group, suggesting that there is efficient sparteine N-oxidation by cytochrome P450 pathways in ALS. This tendency was more marked in 15 patients with ALS aged under 60 years.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00328505
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  • 10
    Electronic Resource
    Electronic Resource
    Atypical motor neuron disease with severe ophthalmoloplegia: a report of two cases (1995)
    Springer
    Journal of neurology 242 (1995), S. 541-546 
    Details
    ISSN: 1432-1459
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00867427
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