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  • 1
    Electronic Resource
    Electronic Resource
    Intermitochondrial septate structures in dystrophic axons (1980)
    Springer
    Acta neuropathologica 52 (1980), S. 105-109 
    Details
    ISSN: 1432-0533
    Keywords: Neuroaxonal dystrophy ; Mitochondria ; Membranes ; Junctions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Intermitochondrial septate structures were found in the dystrophic axons of two cases of infantile neuroaxonal dystrophy. Septate structures were previously seen in some tumors (glioblastomas and Schwannomas) and several organs of vertebrates and invertebrates, but never in human central nervous system (CNS). The structures were studied by transmission and transmission tilt electron microscopy. A proposed model was constructed and X-rayed. Artist's depictions are shown and described. The intermitochondrial septate structures have a periodicity of 120 Å which puts them within the range of those structures previously reported. It was found that our structures are not true junctions, but a complex interdigitation of the outer membranes of the involved mitochondria.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00688007
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  • 2
    Electronic Resource
    Electronic Resource
    Infantile neuroaxonal dystrophy in an albino girl (1985)
    Springer
    Acta neuropathologica 66 (1985), S. 68-71 
    Details
    ISSN: 1432-0533
    Keywords: Infantile neuroaxonal dystrophy ; Albinism ; Spheroids
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We present a cliniconeuropathologic study of infantile neuroaxonal dystrophy (INAD) in a 5-year-old Black girl with albinism. The clinical picture shows progressive psychomotor deterioration, beginning after 1 year of age, with hypotonia, pyramidal signs, optic atrophy, and deafness. Light-microscopic examination of the brain reveals wide distribution of spheroids, cerebellar atrophy, and neuronal loss with astrocytosis. This is the first described case of the combination of INAD with albinism.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00698297
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  • 3
    Electronic Resource
    Electronic Resource
    Topographic heterogeneity of amyloid B-protein epitopes in brains with various forms of neuronal ceroid lipofuscinoses suggesting defective processing of amyloid precursor protein (1990)
    Springer
    Acta neuropathologica 80 (1990), S. 26-34 
    Details
    ISSN: 1432-0533
    Keywords: Neuronal ceroid lipofuscinosis ; Batten disease ; Lipopigment ; Storage disease ; Amyloid precursor protein
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary To verify our hypothesis of defective protease inhibitor domains that are encoded by abnormal processing of amyloid precursor protein (APP) in brains of patients with neuronal ceroid lipofuscinoses (NCL), immunohistochemical and cytochemical studies were performed with monoclonal antibodies (mAbs) directed against various domains of APP. For the studies, 22 autopsy brains were used: 12 with different forms of NCL, and 10 control brains. The staining procedure for the avidin-biotin complex (ABC) technique and the postembedding gold-labelled procedure for electron microscopy (EM) were employed. Of all mAbs used for the study, only mAbs generated against amyloid B-protein bound to neural tissue were affected with NCL. The strongest immunostaining of neurons and of some reactive glial cells was found in brains with the juvenile form of NCL. Only in the infantile form of the disease were some neurons overloaded with storage material weakly immunoreactive. In brains of patients with the adult form of NCL, immunoreactivity was found in affected neurons and in extracellularly deposited material of senile plaques. The results of EM study showed that the immunoreactivity was restricted to lysosomal cytosomes in neural tissue with any form of NCL selectively localized on the curvilinear and fingerprint proteinaceous component of ceroid lipofuscin. Studies performed on control aging brains and Alzheimer's disease (AD) brains confirmed previous observations of immunoreactivity being found diffusely in the protein component of some neurons containing lipopigment. The defective processing of APP in brains with NCL and AD and in ageing brains is discussed. Our present results support the notion of heterogeneity of ceroid lipofuscin storage material in various forms of NCL and underline the hypothesis that abnormalities found in the protease inhibitors or APP in the proteinaceous composition of storage lipopigment could be a key to the unknown etiology of NCL.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294218
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  • 4
    Electronic Resource
    Electronic Resource
    Lectin histochemistry in brains with juvenile form of neuronal ceroid-lipofuscinosis (Batten disease) (1990)
    Springer
    Acta neuropathologica 80 (1990), S. 274-279 
    Details
    ISSN: 1432-0533
    Keywords: Neuronal ceroid-lipofuscinoses ; Batten disease ; Lipid storage disease ; lectin histochemistry ; Inherited neurodegenerative diseases
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Defective ultilization of dolichols in the synthesis of glycoprotein leads to an accumulation of the storage, pigment “ceroid” lipofuscin, containing high-mannose-type glycoconjugates, in brains affected by neuronal ceroid-lipofuscinoses (NCL). We have employed lectin histochemistry to study the distribution of such compounds and the composition of other glycoconjugates in brains of patients with a juvenile form of the disease (JNCL). Concanavalin A detected the high-manose glycoconjugates in all neurons of brains with JNCL, in lipofuscin-containing neurons of aging brains and in some neurons of age-matched control brains. Three other lectins (soybean agglutinin, Peanut agglutinin and Ulex europaeus agglutinin-I) recognized sugar moities in neurons containinglipofuscin in patients only with JNCL and not in age-matched or aging brains. The results led to the conclusion, that the binding pattern of these three lectins may differentiate between storage materials of NCL and aging brains.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294645
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  • 5
    Electronic Resource
    Electronic Resource
    Adult fragile X syndrome (1985)
    Springer
    Acta neuropathologica 67 (1985), S. 289-295 
    Details
    ISSN: 1432-0533
    Keywords: Fragile X ; Synapses ; Dendritic spines ; Macro-orchidism ; Golgi stain
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Fragile X syndrome [fra (X)] is currently accepted as the second most frequent chromosomal disorder associated with developmental disability. Although next to Down syndrome in frequency, no postmortem studies of confirmed adult cases had been reported. The autopsy examination of a 62-year-old, moderately retarded man with the fra (X) syndrome confirmed the preferential involvement of cerebral and testicular structures in this disorder.Dendritic spine abnormalities of the type observed in trisomic chromosomal disorders were associated with synaptic immaturity. Severe testicular hypogonadism accompanied bilateralmacro-orchidism, normal penis, and unilateral hydrocele. Valvular, articular, and testicular interstitial compartments showed normal histochemical staining characteristics for glycoproteins and lipids.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00687814
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  • 6
    Electronic Resource
    Electronic Resource
    The adult and a new late adult forms of neuronal ceroid lipofuscinosis (1992)
    Springer
    Acta neuropathologica 83 (1992), S. 461-468 
    Details
    ISSN: 1432-0533
    Keywords: Neuronal ceroid lipofuscinosis ; Kufs' disease ; Ceroid lipopigment ; Storage disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Three cases of the late adult form of neuronal ceroid lipofuscinosis (NCL) are reported. Two of these are siblings with a late clinical onset at ages 26 and 44 years. The third case, sporadic, has the oldest reported age for the onset of NCL, at 63 years and may be regarded as the first example of the “presenile” form of NCL. The clinical, morphological, histochemical, ultrastructural and genetic features of these three cases are discussed. The literature of the clinicopathological NCL cases with an onset at age of 25 and older is reviewed. The clinical and morphological differences between the late adult form and the presenile form of NCL as well as the difficulties in making the diagnosis are discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00310021
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  • 7
    Electronic Resource
    Electronic Resource
    THE EFFECTS OF KININS AND THEIR INTERACTIONS WITH ADRENERGIC MECHANISMS ON THE BEHAVIOURAL ACTIVITY OF RATS (1974)
    Oxford, UK : Blackwell Publishing Ltd
    Clinical and experimental pharmacology and physiology 1 (1974), S. 0 
    Details
    ISSN: 1440-1681
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: SUMMARY 1. Intraperitoneal administration of kallikrein (50 u/kg) to rats increased the kinin-forming activity of the brain but had no effect on behaviour.2. Injection of bradykinin (1 μg) into a cerebral ventricle slightly decreased the behavioural activity of rats.3. The increase in activity produced by pretreatment with nialamide (200 mg/kg) was greatly potentiated by administration of kallikrein or bradykinin in the doses and by the routes mentioned above.4. Injection of noradrenaline (50 μg) into a cerebral ventricle had no effect on activity in normal rats and slightly increased the activity of rats pretreated with reserpine.5. When noradrenaline was given with kallikrein or bradykinin, activity was markedly increased in normal and reserpinized rats.6. The results suggest that kinins may influence central neurotransmission.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1440-1681.1974.tb00572.x
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  • 8
    Electronic Resource
    Electronic Resource
    Scalar ^1^3C^1^9 F spin-spin couplings between carbonyl carbons and aromatic fluorines in tricarbonylchromium complexes of biphenyl derivatives
    Amsterdam : Elsevier
    Journal of Organometallic Chemistry 423 (1992), S. C13-C15 
    Details
    ISSN: 0022-328X
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
    URL: http://linkinghub.elsevier.com/retrieve/pii/0022-328X(92)83034-F
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  • 9
    Electronic Resource
    Electronic Resource
    Some remarks on the Pd^0/Cu^I-catalysed alkynylation reaction of tricarbonyl(η^6-chlorobenzene)chromium
    Amsterdam : Elsevier
    Journal of Organometallic Chemistry 476 (1994), S. 231-234 
    Details
    ISSN: 0022-328X
    Keywords: Chromium ; Copper ; Palladium
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
    URL: http://linkinghub.elsevier.com/retrieve/pii/0022-328X(94)87087-X
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  • 10
    Electronic Resource
    Electronic Resource
    Effect of angiotensin II and saralasin on motor activity and the passive avoidance behavior of rats
    Amsterdam : Elsevier
    Peptides 9 (1988), S. 475-479 
    Details
    ISSN: 0196-9781
    Keywords: Angiotensin II ; Angiotensin II antagonist ; Motor activity ; Passive avoidance ; Stereotypy
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Chemistry and Pharmacology
    Type of Medium: Electronic Resource
    URL: http://linkinghub.elsevier.com/retrieve/pii/0196-9781(88)90150-7
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