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  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 36 (1981), S. 0 
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract: Recent evidence suggests that a membrane abnormality, expressed in peripheral tissues such as the lymphocyte, may be present in Huntington's disease (HD). Both steady state and time-dependent fluorescence spectroscopic methods were performed on lymphocytes from patients with HD and from age- and sex-matched controls. Lymphocyte membrane dynamics were studied, using fluorescence probes with known specificity for certain membrane areas. These probes included 4-phenylspiro(furan-2(3H)-1′-phthalan)-3,3′-dione (fluorescamine), which binds to surface membrane primary amines, 1–8-anilinonaphthalene sulfonate (1,8-ANS), which inserts at the aqueous-hydrocarbon interface, and 12(9) anthroyl stearate (12(9)AS), which inserts deep in the hydrocarbon core. Steady state fluorescence studies, using fluorescamine, 1–8 ANS, or 12(9)AS, revealed no significant difference between intact HD and control lymphocytes. Time-dependent energy-transfer polarization studies for fluorescamine (tryptophan → fluorescamine) did, however, reveal a slower time decay of I1/I for intact HD lymphocytes as compared with controls. This time-dependent difference may relate to alterations in translational (lateral) and angular mobilities of membrane donors (tryptophan) relative to acceptors (fluorescamine) in intact HD lymphocytes. Such observations support the concept of a membrane abnormality in HD.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 33 (1979), S. 0 
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract– 8-Anilino-1-naphthalene sulfonic acid (ANS), a fluorescent probe specific for membrane polar-apolar interfaces, has been used to investigate differences in corrected excitation, emission and scanned excitation polarization spectra of intact age, sex and passage number matched Huntington's disease (HD) and normal fibroblasts grown in cell culture. At passage number 4, the HD fibroblasts, compared to normals, exhibited a 30-fold increase in excitation and emission intensity, a 30 nm blue shift of the emission maximum and a 0.18 polarization unit increase. These findings suggest that the ANS molecule experiences either increased binding sites or an increased quantum yield, a more nonpolar environment, and a more restricted motion in HD fibroblasts. Continued subculture at higher passages (10) showed a change of these findings towards normals. These fluorescent studies support the concept that a membrane abnormality is present in HD.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Annals of the New York Academy of Sciences 305 (1978), S. 0 
    ISSN: 1749-6632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Natural Sciences in General
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-2072
    Keywords: Denervation ; Dihydroxytryptamines ; Muscle spasms ; Myoclonus ; Serotonin ; Super-sensitivity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Muscle twitches and autonomic changes were induced by systemic injections of L-5-hydroxytryptophan (5-HTP) or the serotonin agonist 5-methoxy-N,N-dimethyltryptamine (5-MeO-DMT) in rats previously lesioned with intracranial 5,7-dihydroxytryptamine (5,7-DHT) after desmethylimipramine. Movements were recorded sensitively and continuously by an electronic activity monitor. Spontaneous locomotor activity was strongly reduced after 5-HTP in both intact and lesioned rats, so that electronically recorded activity correlated very closely with disordered jerking movements scored by a behavioral rating scale. This myoclonus was dependent on the doses of 5-HTP and of 5,7-DHT and was strongly inhibited by serotonin antagonists. In lesioned rats, myoclonus occurred with unaltered activity of monoamine oxidase (MAO) and after only small increases in serotonin levels after 5-HTP, but even large increases in availability of serotonin in intact rats, or strong inhibition of serotonin uptake failed to induce myoclonus unless MAO was first inhibited. The response to 5-HTP in lesioned rats was attenuated by repeated injections of 5-HTP or 5-MeO-DMT. This decreased response was in turn blocked by repeated doses of a serotonin antagonist, but appeared not to be due to altered metabolism of 5-HTP or of serotonin; repeated pretreatment with cyproheptadine potentiated the myoclonic response to 5-HTP after DHT. Changes in postsynaptic receptors may be important in the behavioral supersentivity following 5,7-DHT, and restitution of serotonin or stimulation of its receptors after presynaptic denervation may suppress an evolving supersensitivity at receptive postsynaptic membranes.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 31 (1975), S. 1-12 
    ISSN: 1432-0533
    Keywords: Lissencephaly ; Pachygyria ; Neuronal Migration ; Laminar Necrosis ; Hypoxia ; Perfusion Failure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The architectonic features of abnormal cerebral cortex in a brain with lissencephaly and pachygyria suggest than neuronal migration was interrupted by cortical and subcortical laminar necrosis in the fourth fetal month. The severest cortical abnormality lies in the distal perfusion fields of the major cerebral arteries, while the normal areas are located in the proximal perfusion fields. These architectonic and topographic features suggest that intra-uterine hypoxia or perfusion failure may be a pathogenetic mechanism leading to lissencephaly and pachygyria.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-0533
    Keywords: Porencephaly ; Perfusion failure ; Circle Willis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An infant with multiple congenital anomalies was found at autopsy to have a porencencephalic defect on the ventral surface of the left frontal lobe. The intracranial defect was seen in association with an anomalous configuration of the circle of Willis. The zone of tissue destruction corresponded to the vascular territory of the anterior choroidal and lenticulo-striate branches of the proximal middle cerebral arteries, which were absent on the left. The developmental anomaly of the circle of Willis may have predisposed to tissue destruction by compromising cerebral perfusion at midgestation, a stage of rapid brain growth.
    Type of Medium: Electronic Resource
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