Library

X
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
    Enzyme replacement treatment for Tay-Sachs disease brain cells in culture utilizing Concanavalin A-mediated hexosaminidase A uptake: Biochemical and morphological evidence of GM2 mobilization (1980)
    Springer
    Acta neuropathologica 50 (1980), S. 9-17 
    Details
    ISSN: 1432-0533
    Keywords: Enzyme replacement ; Hex A ; TSD ; Con A ; Brain cells ; GM2
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary When Concanavalin A (Con A) is bound to the cell membrane, it functions as an artificial enzyme receptor, mediating the binding and intracellular incorporation of significant amounts of exogenous hexosaminidase A (Hex A) into Tay-Sachs disease (TSD) glial cells. The treated cells retained almost 50% of incorporated Hex A activity after 3 days incubation in Hex A free medium. Hex A was released from Con A within the cell and was available as free enzyme. Biochemical analysis of gangliosides in Con A and Hex A treated cells depicted a greater than 50% reduction in stored GM2 ganglioside and a fourfold reduction in GM2label (14C) when compared to controls. Ultrastructural evidence of GM2 breakdown is presented which supports the biochemical and labeling studies.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00688529
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 2
    Electronic Resource
    Electronic Resource
    Electron microscopic and enzyme histochemical studies of cerebellum, ocular and skeletal muscles in chronic progressive ophthalmoplegia with cerebellar ataxia (1973)
    Springer
    Acta neuropathologica 23 (1973), S. 300-312 
    Details
    ISSN: 1432-0533
    Keywords: Chronic Progressive Ophthalmoplegia ; Cerebellar Ataxia ; Electron Microscopy ; Enzyme Histochemistry ; Mitochondria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Electron microscopic and enzyme histochemical studies were performed on the cerebellum and the ocular and deltoid muscles from a 38 year old woman who developed bilateral ptosis at the age of nine years. Histologically the cerebellum appeared normal. The biopsies of three ocular muscles showed varying sizes of muscle fibers which were rounded and contained increased numbers of subsarcolemmal nuclei. The deltoid muscle stained by hematoxylin and eosin appeared normal, but the trichrome stain showed increased numbers of red granules within the sarcolemma corresponding ultrastructurally to increased numbers of abnormal mitochondria. These abnormal mitochondria displayed increased reaction products with LDH, NADH and SDH preparations, while the muscle gave normal reaction in phosphorylase, PAS and myosin ATP preparations. Chemical studies on the cerebellum showed normal proteolipids, glycolipids and phospholipids. Ultrastructurally, the cerebellum, the myofibers of three ocular muscles and the deltoid muscle exhibited abnormal mitochondria which showed peculiarly arranged circular cristae. They frequently contained paracrystalline structures which consisted of individual tubules arranged in a helical pattern. Frequently, the abnormal mitochondria were replaced by dense rectangular inclusions and occasionally showed complete transition to crystalline structures.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00687459
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 3
    Electronic Resource
    Electronic Resource
    Electron miscroscopic and enzyme histochemical studies of the cerebellum in spongy degeneration (1972)
    Springer
    Acta neuropathologica 20 (1972), S. 22-31 
    Details
    ISSN: 1432-0533
    Keywords: Spongy Degeneration ; Cerebellum ; Electron Microscopy ; Enzyme Histochemistry ; Mitochondria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Electron microscopic and enzyme histochemical studies were performed on the cerebellum from a 9 month old Jewish boy with spongy degeneration. Histologically, the main pathological changes were noted in the Purkinje cell layer, the deeper areas of the granular cell layers and the subcortical white matter. Ultrastructurally, multiple vacuoles were present within the swollen cytoplasm and processes of protoplasmic astrocytes in the cortex, while in the subcortical white matter vacuoles were observed within splitting myelin lamellae as well as within astrocytes. There were also abnormal mitochondria within swollen protoplasmic astrocytic cytoplasm and processes which in ATPase preparations showed little or no reaction product. However, the fibrillary astrocytes were not swollen and contained intact mitochondria which showed normal reaction product in ATPase preparations. Since the myelin changes are known to be nonspecific and secondary to abnormal fluid accumulation, the characteristic distribution of the multiple vacuoles in the central nervous system in this disorder seems primarily to be related to swelling of the protoplasmic astrocytes.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00687899
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 4
    Electronic Resource
    Electronic Resource
    Ceramide hexosides in niemann-pick disease brain (1976)
    Springer
    Journal of neurology 213 (1976), S. 251-255 
    Details
    ISSN: 1432-1459
    Keywords: Niemann-Pick disease ; Ceramide trihexoside ; Asialo-GM2 ; Gangliosides ; Cerebroside ; Hexosylceramides
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Zusammenfassung In normalem Gehirngewebe und in Gehirngewebe einer Niemann-Pickschen Erkrankung vom Typus A wurden Glykosphingolipide unter Verwendung der Dünnschichtchromatographie und der Flüssig-Gaschromatographie bestimmt. Bei der Niemann-Pickschen Erkrankung fand sich eine Zunahme der Ceramid-Trihexoside, die durch Digalactosylglucosylceramide bedingt war. Auch das Glucosylcerebrosid war vermehrt.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00312874
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 5
    Electronic Resource
    Electronic Resource
    Gangliosides in SV-40-transformed cells derived from Tay-Sachs disease fetal brain (1989)
    Springer
    Metabolic brain disease 4 (1989), S. 87-93 
    Details
    ISSN: 1573-7365
    Keywords: Tay-Sachs disease ; GM2 ; ganglioside ; brain cell cultures ; SV-40 transformed
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A human glial brain cell line derived from a Tay-Sachs disease fetal cerebellum was transformed with SV-40 virus in order to obtain a transformed brain cell line which reflected the characteristics of the disease. It was shown that the transformed TSD cell line maintained an elevated level of GM2 which was similar to that shown by the nontransformed precursor. In addition, the TSD transformed line lacked hexosaminidase A.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00999386
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
Close ⊗
This website uses cookies and the analysis tool Matomo. More information can be found here...