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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
    Motor neuron disease with multi-system involvement presenting as tetraparesis, ophthalmoplegia and sensori-autonomic dysfunction (1994)
    Springer
    Acta neuropathologica 88 (1994), S. 193-200 
    Details
    ISSN: 1432-0533
    Keywords: Motor neuron disease ; Amyotrophic lateral sclerosis ; Extrapyramidal system Reticular formation ; Ophthalmoparesis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We carried out a postmortem examination on two Japanese patients, 64- and 80-year-old men whose survival was prolonged with an artificial respirator. They had no family history of neuropsychiatric disorders and were suspected, clinically, as having a motor neuron disease that differed from amyotrophic lateral sclerosis (ALS). As well as upper and lower motor neuron impairment, they showed a variety of symptoms, such as sensory disturbances, hypohidrosis, impotence, ophthalmoparesis and/or atonic neurogenic bladder, and their protein content in cerebrospinal fluid was elevated markedly. Pathological examination revealed the following extensive nervous system involvement: (1) the upper and lower voluntary motor systems, including the IIIrd, IVth and VIth cranial nerve nuclei: (2) the reticular formation and its major afferent pathways; (3) the vestibulospinal and tectospinal systems; (4) the spinocerebellar system and the exteroceptive somatic afferent pathways; (5) the dentatorubral and pallidoluysian system; and (6) the substantia nigra, locus ceruleus and intermediolateral and Onufrowicz's nuclei. Neither Bunina bodies, Lewy body-like hyaline inclusions nor ubiquitin immunoreactive skein-like structures were observed. The distribution of the lesions was quite different from that in patients with ALS and the other known related diseases. Recently, seven autopsied cases with clinical and histopathological similarities to our patients have been reported in Japan. Our conclusion is that our two and these seven patients should be classified as having a new motor neuron disease entity, which can be is differentiated from ALS.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00293393
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  • 5
    Electronic Resource
    Electronic Resource
    Motor neuron disease with multi-system involvement presenting as tetraparesis, ophthalmoplegia and sensori-autonomic dysfunction (1994)
    Springer
    Acta neuropathologica 88 (1994), S. 193-200 
    Details
    ISSN: 1432-0533
    Keywords: Key words Motor neuron disease ; Amyotrophic lateral sclerosis ; Extrapyramidal system ; Reticular formation ; Ophthalmoparesis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We carried out a postmortem examination on two Japanese patients, 64- and 80-year-old men whose survival was prolonged with an artificial respirator. They had no family history of neuropsychiatric disorders and were suspected, clinically, as having a motor neuron disease that differed from amyotrophic lateral sclerosis (ALS). As well as upper and lower motor neuron impairment, they showed a variety of symptoms, such as sensory disturbances, hypohidrosis, impotence, ophthalmoparesis and/or atonic neurogenic bladder, and their protein content in cerebrospinal fluid was elevated markedly. Pathological examination revealed the following extensive nervous system involvement: (1) the upper and lower voluntary motor systems, including the IIIrd, IVth and VIth cranial nerve nuclei: (2) the reticular formation and its major afferent pathways; (3) the vestibulospinal and tectospinal systems; (4) the spinocerebellar system and the exteroceptive somatic afferent pathways; (5) the dentatorubral and pallidoluysian systems; and (6) the substantia nigra, locus ceruleus and intermediolateral and Onufrowicz's nuclei. Neither Bunina bodies, Lewy body-like hyaline inclusions nor ubiquitin immunoreactive skein-like structures were observed. The distribution of the lesions was quite different from that in patients with ALS and the other known related diseases. Recently, seven autopsied cases with clinical and histopathological similarities to our patients have been reported in Japan. Our conclusion is that our two and these seven patients should be classified as having a new motor neuron disease entity, which can be is differentiated from ALS.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00293393
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  • 6
    Electronic Resource
    Electronic Resource
    Galactosialidosis: neuropathological findings in a case of the late-infantile type (1991)
    Springer
    Acta neuropathologica 82 (1991), S. 331-339 
    Details
    ISSN: 1432-0533
    Keywords: Galactosialidosis ; Neuronal storage disease ; Neuropathology ; Ultrastructure ; Neuronal loss
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The neuropathological findings in a 13-year-old Japanese male showing decrease of sialidase and β-galactosidase activities are reported. The patient was the product of normal pregnancy to consanguineous parents. He started to sit at 8 months, stand at 20 months and walk at the age of 2; mental retardation, visual disturbance, cerebellar ataxia, myoclonus and epilepsy developed by the age of 10, and he died at 13. Neuropathological investigation revealed neuronal loss and storage. Severe loss of neurons was observed in the thalamus, globus pallidus, lateral geniculate body, gracile nucleus, Purkinje and retinal ganglion cells. Marked ballooning was seen in the Betz cells and neurons in the basal forebrain, the motor neurons in the cranial nerve nuclei and spinal cord, and in the trigeminal and spinal ganglia. The storage material varied in staining from region to region and from neuron to neuron. Electron microscopic investigation revealed a variety of intracytoplasmic and intranuclear inclusions: membranous cytoplasmic bodies, parallel, wavy-lamellar or tortuous tubular structures, lipofuscin-like irregular-shaped pleomorphic bodies, and cytoplasmic vacuoles with fine granules and lamellar materials. The severity of the neuronal loss did not seem to correlate with the amount of the storage materials, but with the presence of tortuous tubular inclusion.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00296543
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  • 7
    Electronic Resource
    Electronic Resource
    Ataxic polyneuropathy and anti-Pr2 IgMκ M proteinemia (1992)
    Springer
    Journal of neurology 239 (1992), S. 147-151 
    Details
    ISSN: 1432-1459
    Keywords: Neuropathy ; Paraproteinemia ; Cold agglutinin ; Anti-Pr2 antibody ; Ganglioside
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of ataxic neuropathy associated with IgMκ M proteinemia is reported. Double filtration plasmapheresis effectively treated the neuropathy. The IgMκ antibody had anti-Pr2 cold agglutinin activity. We demonstrated reactivities of the IgMκ antibody to sialosyl paragloboside, sialosyl lactosaminyl paragloboside, GT1b, GD1a, GD1b, GM3 and GD3 on high-performance thin-layer chromatography immunostaining and enzyme-linked immunosorbent assay, which is previously unreported antigenic specificity of the M proteins in cases of para proteinemic neuropathy. IgM M protein with anti-Pr2 cold agglutinin activity may play a pathogenetic role in peripheral nerve demyelination, because the target antigens of the M protein are present in myelin and possibly in endothelial cells of the peripheral nervous system.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00833915
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  • 8
    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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  • 9
    Electronic Resource
    Electronic Resource
    S11.9 Molecular mimicry between ganglioside and a bacterium elicits guillain-barré syndrome (1993)
    Springer
    Glycoconjugate journal 10 (1993), S. 294-294 
    Details
    ISSN: 1573-4986
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01210033
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  • 10
    Electronic Resource
    Electronic Resource
    Nitrite, nitrate and cGMP in the cerebrospinal fluid in degenerative neurologic diseases (1995)
    Springer
    Journal of neural transmission 100 (1995), S. 263-267 
    Details
    ISSN: 1435-1463
    Keywords: Amyotrophic lateral sclerosis ; cerebrospinal fluid ; cyclic GMP ; nitric oxide ; Parkinson's disease ; spinocerebellar ataxia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary To investigate whether nitric oxide (NO) plays a role in degenerative neurologic disease (DND), we measured nitrite, nitrate and cyclic GMP in cerebrospinal fluid (CSF) samples from patients with Parkinson's disease (PD), spinocerebellar ataxia (SCA) and amyotrophic lateral sclerosis (ALS). We found no significant change in CSF nitrite, nitrate or cyclic GMP in patients with any DND compared with control values. These results suggest that NO production is preserved in PD, SCA and ALS.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01276464
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