Library

feed icon rss

Your email was sent successfully. Check your inbox.

An error occurred while sending the email. Please try again.

Proceed reservation?

Export
  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 30 (1978), S. 0 
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract— Biochemical analysis of the leukodystrophy brain from a case of Pelizaeus-Merzbacher disease, classical type, was performed. A decrease in the amount of solid material present was found. The lyophilized brain weight was reduced to 76% of normal with a slightly greater decrease in the amount of extractable lipid. Total myelin was diminished to 7% of normal. Among specific lipids plasmalogens were present in slightly lowered amounts. Cerebrosides and sulphatides were drastically reduced to 8% of normal, whereas sphingomyelin was less severely affected. Fatty acids from phospholipids were close to normal, only enols being slightly diminished. Analysis of pure cerebrosides and sulphatides revealed that the a-hydroxylated compounds as well as very long chain fatty acids (over C18, especially C23 to C26) were greatly reduced. For chain lengths over C18, the ratio of leukodystrophy fatty acid to normal fatty acid was close to 10%. The defect in very long chain fatty acids is estimated at 99.2% in total brain.Thus, we have found a marked decrease in the amount of very long chain fatty acids and a less marked decrease in sphingolipids. The reduced amount of these acids appears to be partially offset by an increase in the amount of medium-chain fatty acids in sphingolipids. We conclude that one aspect of Pelizaeus-Merzbacher disease may be a defect in the synthesis of myelin very long chain fatty acids (as these acids are far much reduced than any other myelin molecule).
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 2
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 24 (1975), S. 0 
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 3
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Journal of neurochemistry 32 (1979), S. 0 
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The brains of Quaking and littermate control mice were fractionated by differential and density gradient centrifugation into soluble, microsomal, myelin and related (SN 4) fractions. There were no apparent differences in protein composition between any Quaking and control fraction with the exception of myelin and SN 4. Analysis of CNP activity indicated that in Quaking animals a high proportion of the total activity was localized in microsomal fractions, while in controls a large percentage of activity was found in myelin and SN4; in contrast, there were no marhcd differences in the distribution of AChE activity between Quaking and control fractions.The yield of myelin isolated from Quaking animals was 3.6%, of that from controls by electron microscopy myelin fractions from both Quaking and controls consisted of compact myelin whorls. Zonal centrifugation on continuous sucrose gradients demonstrated that both control and Quaking myelin was distributed in a bell-shaped mode with peak densities at 0.66 0.68 and 0.71-0.75 M-sucrose, respectively. The specific activity of CNP was generally lower in mutant subfractions than in controls. Protein analysis revealed that there were similar qualitative trends between light and heay myelin subfractions from both mutant and control animals, although the levels of proteolipid and small basic proteins were substantially lower in all Quaking fractions. These results indicate that. although all mutant myelin subfractions are compositionally abnormal, the type of particle heterogeneity in Quaking myelin is similar to that observed in controls.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 45 (1979), S. 133-140 
    ISSN: 1432-0533
    Keywords: Alexander's disease ; Rosenthal's fibers ; Peripheral Nerve ; Electron microscopy ; Neurochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The authors report the results of a cerebral and of a neuromuscular biopsies and of the autopsy findings in another infantile case of Alexander's disease in a girl. They review the 17 previously reported cases of this disease and the various etiopathogenic hypotheses mentioned. The presence of numerous, sometimes abnormal enlarged mitochondria and of abundant membranous cytoplasmic bodies in the astrocytic cytoplasm seems to be unreported elsewhere. Peripheral nerve changes are mentioned for the first time.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 5
    Electronic Resource
    Electronic Resource
    Weinheim : Wiley-Blackwell
    Zeitschrift für anorganische Chemie 418 (1975), S. 167-170 
    ISSN: 0044-2313
    Keywords: Chemistry ; Inorganic Chemistry
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology
    Description / Table of Contents: Esters of Stibonous AcidAntimony tribromide reacts with t-butylmagnesium chloride in the ratio of 1:1 at low temperature under formation of t-butyldibromostibine. The reaction with sodium alcoholates yields t-butyldialkoxistibines. Interchange reactions with 1,2-diols give 2-t-butyl-1,3,2-dioxastiboles and -stibolanes, which do not form coordination polymeres.
    Notes: Antimontribromid reagiert mit t-Butylmagnesiumchlorid im Verhältnis 1:1 bei tiefen Temperaturen unter Bildung von t-Butyldibromstibin. Durch Umsetzung mit Natriumalkoholaten gelangt man zu t-Butyldialkoxystibinen. Umesterungsreaktionen letzterer mit 1,2-Diolen führen zu 2-t-Butyl-1,3,2-dioxastibolen und -stibolanen. Diese liegen im Gegensatz zu den bisher bekannten Verbindungen dieses Typs nicht als Koordinationspolymere vor.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
  • 6
    Electronic Resource
    Electronic Resource
    Weinheim : Wiley-Blackwell
    Zeitschrift für anorganische Chemie 418 (1975), S. 279-286 
    ISSN: 0044-2313
    Keywords: Chemistry ; Inorganic Chemistry
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology
    Description / Table of Contents: Esters of Stibonic AcidSymmetric spirocyclic esters of stibonic acid are obtained by esterification of phenylstibonic acid with 1,2-diols in the ratio of 1:2. The addition of o-quinones to 2-organo- and 2-halogeno-1,3,2-dioxastibolanes or methyldialkoxystibines yields unsymmetrical spirocyclic respectively cyclic esters of stibonic acid. The oxidation of t-butyl substituted stibines and cyclic esters of t-butylstibonous acid is not successful. Antimony(III) compounds are formed under decomposition.
    Notes: Symmetrische spirocyclische Stibonsäureester erhält man durch Veresterung von Phenylstibonsäure mit 1,2-Diolen im Verhältnis 1:2. Die Addition von o-Chinonen an 2-Organo- und 2-Halogeno-1,3,2-dioxastibolane oder Methyldialkoxystibine führt zu unsymmetrischen spirocyclischen bzw. cyclischen Stibonsäureestern. Die Oxydation von t-butylsubstituierten Stibinen und cyclischen Stibonigsäureestern gelingt nicht. Man erhält Verbindungen des dreiwertigen Antimons unter Abspaltung der t-Butylgruppe.
    Type of Medium: Electronic Resource
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
Close ⊗
This website uses cookies and the analysis tool Matomo. More information can be found here...