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  • 1
    Electronic Resource
    Electronic Resource
    The significance of cytoplasmic inclusions in late infantile and juvenile amaurotic idiocy (1973)
    Springer
    Acta neuropathologica 23 (1973), S. 32-42 
    Details
    ISSN: 1432-0533
    Keywords: Juvenile Amaurotic Idiocy ; Multilamellar, Crescentic, “fingerprint” ; Inclusions ; Lipofuscin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The electron microscopic findings of cerebral biopsies from two siblings with late infantile-juvenile amaurotic idiocy are presented. One patient had a mixture of “fingerprint” and multilamellar inclusions, often in the same cytosome. The second patient had only “fingerprint” inclusions. Electron microscopic findings in autopsied tissues from two other cases of late infantile-juvenile amaurotic idiocy with multilamellar cytosomes are also presented. Evolution of the multilamellar cytosomes into lipofuscin was not observed. We concluded that cases of late infantile-juvenile amaurotic idiocy with multilamellar and “fingerprint” inclusions should be distinguished from cases with lipofuscin.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00689002
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  • 2
    Electronic Resource
    Electronic Resource
    Familial spongy degeneration of the central nervous system (Van Bogaert-Bertrand disease) (1969)
    Springer
    Acta neuropathologica 12 (1969), S. 103-115 
    Details
    ISSN: 1432-0533
    Keywords: Spongy Degeneration of the CNS ; Van Bogaert-Bertrand-Disease ; Electron Microscopy ; Astrocytic Mitochondria ; Abnormal Cerebral Edema
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wird über licht- und elektronenoptische Untersuchungen an Muskel-und Hirnbiopsien eines Falles von spongiöser Degeneration des ZNS berichtet. Die in der grauen und weißen Hirnsubstanz enthaltenen Vacuolen entsprechen a) Spalten in den Markscheiden infolge Aufsplitterung an der intraperiodischen Linie und b) geschwollenen Astrocytenperikaryen und-fortsätzen. Ungewöhnliche Mitochondrien mit Gehalt an kristallinem Material fanden sich nur in Astrocyten. Die ultrastrukturellen Befunde entsprechen denen des Hirnödems. Es wird angenommen, daß die Astroglia eine primäre Rolle in der Flüssigkeitsansammlung spielt, während die Markscheidenschwellung als eine Sekundärläsion aufgefaßt wird. Die mögliche Bedeutung abnormer Astrocyten-Mitochondrien wird diskutiert.
    Notes: Summary Light and electron microscopy study of skeletal muscle and cerebral biopsies from a case of spongy degeneration of central nervous system is reported. The multiple vacuoles present in cerebral gray and white matter correspond to (a) clefts within myelin sheaths resulting from splitting at the intraperiod line and (b) swollen astrocytic perikarya and processes. Unusual mitochondria containing crystalline-like material were observed only in astrocytes. The ultrastructural findings are consistent with cerebral edema. It is suggested that the astrocytes play a primary role in the fluid accumulation while the myelin swelling is a secondary lesion. The possible role of the abnormal astrocytic mitochondria is discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00692500
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  • 3
    Electronic Resource
    Electronic Resource
    Accumulation of smooth cisterns, multivesicular bodies and “zebra” bodies in neurons (1971)
    Springer
    Acta neuropathologica 18 (1971), S. 132-143 
    Details
    ISSN: 1432-0533
    Keywords: Psychomotor Retardation ; Smooth Cisterns ; Zebra Bodies ; Storage Disorder ; Mode of Formation of Abnormal Cytosomes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The ultrastructural and biochemical study of cerebral and liver biopsies from a two-year-old boy with 20 month history of psychomotor retardation, hypotonia and hyporeflexia is presented. Although the case cannot be considered, at the present time, as a separate entity in view of the failure of the biochemical studies of identifying the stored material, the ultrastructural features, which include clustered agranular cisterns of endoplasmic reticulum and numerous multivesicular bodies and “zebra” bodies in neurons, indicate a storage disorder morphologically different from those reported to date. In the present case, morphological and cytochemical findings suggest that the agranular endoplasmic reticulum may give trise to multivesicular bodies which, in turn, may become dense bodies and “zebra” bodies.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00687601
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  • 4
    Electronic Resource
    Electronic Resource
    Neurodegeneration Chaperoning brain diseases (1998)
    [s.l.] : Macmillan Magazines Ltd.
    Nature 392 (1998), S. 23-24 
    Details
    ISSN: 1476-4687
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Notes: [Auszug] A common feature of several neurodegenerative disorders — including Alzheimer's and Parkinson's diseases — is a pathogenetic mechanism which is similar to that proposed for prion diseases (Table 1). The proteins involved have poorly structured native conformations that can be ...
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1038/32049
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  • 5
    Electronic Resource
    Electronic Resource
    Molecular assessment of the potential transmissibilities of BSE and scrapie to humans (1997)
    [s.l.] : Macmillan Magazines Ltd.
    Nature 388 (1997), S. 285-288 
    Details
    ISSN: 1476-4687
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Notes: [Auszug] More than a million cattle infected with bovine spongiform encephalopathy (BSE) may have entered the human food chain. Fears that BSE might transmit to man were raised when atypical cases of Creutzfeldt–Jakob disease (CJD), a human transmissible spongiform encephalopathy (TSE), emerged in ...
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1038/388285a0
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  • 6
    Electronic Resource
    Electronic Resource
    Binding of Bodian's Silver and Monoclonal Antibodies to Defined Regions of Human Neurofilament Subunits: Bodian's Silver Reacts with a Highly Charged Unique Domain of Neurofilaments (1986)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 46 (1986), S. 0 
    Details
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract: Cleavage at cysteine and chymotrypsin digestion were applied to two human neurofilament (NF) sub-units, low- and high-molecular-weight NF (NF-L and NF-H), to locate the regions reacting with Bodian's silver stain and with several monoclonal antibodies, including NF-specific antibodies and one that recognizes all intermediate filaments (anti-IFA). Our findings indicate that whereas anti-IFA recognizes the highly conserved rod domain, all the NF-specific antibodies, as well as Bodian's silver, react with the carboxy-terminal tailpiece of NF subunits. The silver binding sites in NF-L are located in a carboxy-terminal 12-Kd chymotrypsin fragment, a highly charged, unique domain of NF.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1471-4159.1986.tb12977.x
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  • 7
    Electronic Resource
    Electronic Resource
    Amyloid Precursor Protein Metabolism in Primary Cell Cultures of Neurons, Astrocytes, and Microglia (1996)
    Oxford, UK : Blackwell Science Ltd
    Journal of neurochemistry 66 (1996), S. 0 
    Details
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract: Amyloid precursor protein (APP) gives rise by proteolytic processing to the amyloid β peptide (Aβ) found abundantly in cerebral senile plaques of individuals with Alzheimer's disease. APP is highly expressed in the brain. To assess the source of cerebral Aβ, the metabolism of APP was investigated in the major cell types of the newborn rat cerebral cortex by pulse/chase labeling and immunoprecipitation of the APP and APP metabolic fragments. We describe a novel C-terminally truncated APP isoform that appears to be made only in neurons. The synthesis, degradation, and metabolism of APP were quantified by phosphorimaging in neurons, astrocytes, and microglia. The results show that although little APP is metabolized through the amyloidogenic pathways in each of the three cultures, neurons appear to generate more Aβ than astrocytes or microglia.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1471-4159.1996.66062300.x
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  • 8
    Electronic Resource
    Electronic Resource
    Immunochemical Characterization of Antisera to Rat Neurofilament Subunits (1981)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 37 (1981), S. 0 
    Details
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract: Antisera raised to the 68,000, 145,000 and 200,000 molecular weight subunits of rat neurofilaments were used for immunochemical staining of polypeptides separated by one- and two-dimensional gel electrophoresis. It was found that each antiserum reacts intensely with its corresponding neurofilament subunit and weakly with the other two subunits. All the antisera also react with a polypeptide of molecular weight 57,000 present in neurofilament-rich preparations from both rat spinal cord and peripheral nerve. This polypeptide is different from either tubulin or vimentin and may represent a neurofilament breakdown product, since it varied in amount from preparation to preparation. The three antisera also reacted with the polypeptide subunits of chicken and goldfish neurofilament despite the considerable difference in molecular weight between these subunits and those of mammalian neurofilament. Key Words: Neurofilaments–Antibodies–Immunochemical. Autilio-Gambetti L. et al. Immunochemical characterization of antisera to rat neurofilament subunits. J. Neurochem. 37, 1260-1265(1981).
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1471-4159.1981.tb04676.x
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  • 9
    Electronic Resource
    Electronic Resource
    Intermediate Filaments of Schwann Cells (1982)
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 38 (1982), S. 0 
    Details
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract: Intermediate filaments were prepared from distal stumps of rabbit sciatic nerve 5 weeks after nerve section, at which time Schwann cells account for 85–90% of the cell area. A polypeptide of molecular weight 58,000 was the main component of this fraction. An antiserum raised in guinea pig against this polypeptide stained all cells present in the distal stump, as well as Schwann cells and 3T3 cells in culture. The identity of the molecular weight 58,000 polypeptide obtained from distal stumps with vimentin was proved with one and two-dimensional sodium dodecyl sulfate pol yacrylamide gel electrophoresis and with immunoautoradiography. It is concluded that the intermediate filament subunit of undifferentiated Schwann cells is vimentin. The possibility that Schwann cells in normal nerve may have another type of intermediate filament besides vimentin cannot be ruled out.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1471-4159.1982.tb08698.x
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  • 10
    Electronic Resource
    Electronic Resource
    Biochemical fingerprints of prion diseases: scrapie prion protein in human prion diseases that share prion genotype and type (2005)
    Oxford, UK : Blackwell Science Ltd
    Journal of neurochemistry 92 (2005), S. 0 
    Details
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The phenotype of human prion diseases is influenced by the prion protein (PrP) genotype as determined by the methionine (M)/valine (V) polymorphism at codon 129, the scrapie PrP (PrPSc) type and the etiology. To gain further insight into the mechanisms of phenotype determination, we compared two-dimensional immunoblot profiles of detergent insoluble and proteinase K-resistant PrP species in a type of sporadic Creutzfeldt-Jakob disease (sCJDMM2), variant CJD (vCJD) and sporadic fatal insomnia (sFI). Full-length and truncated PrP forms present in the insoluble fractions were also separately analyzed. These three diseases were selected because they have the same M/M PrP genotype at codon 129 and the same type 2 PrPSc, but different etiologies, also sCJDMM2 and sFI are sporadic, whereas vCJD is acquired by infection. We observed minor differences in the PrP detergent-insoluble fractions between sCJDMM2 and vCJD, although both differ in the corresponding fractions from sFI. We detected more substantial heterogeneity between sCJDMM2 and vCJD in the two-dimensional blots of the proteinase K-resistant PrP fraction suggesting that different PrP species are selected for conversion to proteinase K-resistant PrP in sCJDMM2 and vCJD. These differences are mostly, but not exclusively, due to variations in the type of the N-linked glycans. We also show that the over-representation of the highly glycosylated forms distinctive of the proteinase K-resistant PrPSc of vCJD in one-dimensional blots is due to differences in both the amount and the natures of the glycans. Overall, these findings underline the complexity of phenotypic determination in human prion diseases.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1471-4159.2004.02859.x
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