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  • 1
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Congenital cataracts facial dysmorphism neuropathy (CCFDN) syndrome (OMIM 604168) is an autosomal recessive developmental disorder that occurs in an endogamous group of Vlax Roma (Gypsies; refs. 1–3). We previously localized the gene associated with CCFDN to 18qter, where a conserved ...
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Naturwissenschaften 57 (1970), S. 44-44 
    ISSN: 1432-1904
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Chemistry and Pharmacology , Natural Sciences in General
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 99 (2000), S. 539-546 
    ISSN: 1432-0533
    Keywords: Key words Diabetic neuropathy ; Collagen ; Extracellular matrix ; Nerve regeneration
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The pattern of collagenisation in peripheral nerve in diabetic polyneuropathy was examined in nerve biopsy specimens from patients with diabetic polyneuropathy in comparison with organ donor control nerves and disease controls (other neuropathies). There was increased endoneurial collagenisation both in the diabetic polyneuropathy cases and the disease controls, this predominantly involving types I and III. Type II collagen was not detected in organ donor control nerves or in the diabetic and the disease control nerves. There was a relative increase in type VI collagen in the endoneurium in the diabetic nerves immediately surrounding groups of Schwann cells. This was not a feature in the other neuropathies. The quantity of types IV, V and VI collagen was increased around the endoneurial microvessels in the diabetic patients and, to a lesser extent, in those with hereditary motor and sensory neuropathy (HMSN). Increased deposition of types IV and V collagen was observed in the perineurium in the diabetic nerves, the latter being most evident in the innermost lamellae where the amount of laminin was possibly also increased. The diameter of the general endoneurial collagen fibrils was greater in the diabetic nerves, although this was not more than in a disease control (HMSN). The collagen fibrils that were present within the basal laminal tubes that had surrounded degenerated myelinated fibres in the diabetic nerves, and those within the onion bulbs of the HMSN cases, were of the normal endoneurial calibre. The expression of laminin by Büngner bands in diabetic neuropathy did not differ from that in disease control nerves, nor were any differences detected for fibronectin. Whether the changes observed are important for the impaired regenerative capacity in diabetic neuropathy requires further investigation.
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 11 (1968), S. 93-102 
    ISSN: 1432-0533
    Keywords: Diabetic Neuropathy ; Hypertrophic Changes ; Nerve Biopsy ; Electron Microscopy ; Segmental Demyelination
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Beobachtungen an Nervenbiopsien bei 10 aufeinanderfolgenden Patienten mit diabetischer Neuropathie wurden unternommen. 1 Patient wies die für eine hypertrophische Neuropathie typischen licht-und elektronenmikroskopischen Veränderungen auf. 5 zeigten typische hypertrophische Veränderungen, die aber nur bei elektronenmikroskopischer Untersuchung sichtbar waren; bei weiteren wurden ähnliche geringe Veränderungen entdeckt. Es wird angenommen, daß diese Veränderungen durch segmentale Demyelinisation verursacht wurden.
    Notes: Summary Observations have been made on 10 consecutive nerve biopsies from patients with diabetic neuropathy. 1 patient showed the typical appearances of hypertrophic neuropathy on light and electron microscopy. 5 displayed typical hypertrophic changes visible only on electron microscopy and minor abnormalities of a similar nature were seen in 2 others. It was considered that they were likely to have resulted from recurrent segmental demyelination.
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 14 (1969), S. 237-249 
    ISSN: 1432-0533
    Keywords: Electron Microscopy ; Wallerian Degeneration ; Nodal Changes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wird über ultrastrukturelle Veränderungen in den Ranvierschen Knoten des N.suralis der Ratte im Laufe der Wallerschen Degeneration berichtet. Die Untersuchungen erfolgten 12 und 120 Std nach einer örtlichen Quetschungsverletzung. Die ersten bemerkbaren Veränderungen finden sich im Axon. Nodale und paranodale Anhäufungen von Mitochondrien, multivesikulären und lamellären Körpern, wie auch kleinen blasen- und röhrenartigen Bildungen sind teilweise in den Knoten sichtbar und am deutlichsten nach 24–36 Std erkennbar. Gleichzeitig erfolgt eine Aufsplitterung der Neurofilamente und Neurotubuli, die ihre Liniengestaltung verlieren und sich zusammenballen. Die Zone der erhöhten Dichte gerade unterhalb des nodalen Axolemmas bleibt erhalten. Veränderungen im Myelin beginnen etwas später und bestehen in einem vesikulären Verfall der Endomyelinlamellen und einer Trennung der Endomyelinschlaufen vom Axolemma durch Schwannzellenfortsätze. Dieser Vorgang schien mit einem Zurückziehen des Myelins vom Knoten im Zusammenhang zu stehen. Schwannzellenfortsätze erstrecken sich auch so weit, daß sie das nodale Axon bedecken, wobei sie die nodalen Schwannzellenfortsätze vom Axolemma trennen. Das Endstadium ist die Unterbrechung des nodalen Axons und die Verschmelzung der Myelinenden als Teil der Ovoidbildung.
    Notes: Summary Observations are reported on the ultrastructural alterations at the nodes of Ranvier in the rat sural nerve during the course of Wallerian degeneration. These were examined between 12 and 120 hours after a localized crush injury. The earliest detectable changes are in the axon. Nodal and paranodal accumulations of mitochondria, multivesicular bodies, lamellar bodies and small vesicular and tubular profiles are seen at a proportion of the nodes and are most evident at 24–36 hours. Concomitantly with this, the neurofilaments and neurotubulus fragment, lose their alignment and clump together. The zone of increased density just beneath the nodal axolemma is preserved. Changes in the myelin begin slightly later and consist of vesicular breakdown of the terminal myelin lamellae, and separation of the terminal myelin loops from the axolemma by Schwann cell processes. The latter event appeared to be associated with retraction of the myelin from the node. Schwann cell processes also extend to cover the nodal axon, separating the Schwann cell nodal processes from the axolemma. The final stage is the interruption of the nodal axon and the fusion of the ends of the myelin as part of ovoid formation.
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  • 6
    ISSN: 1432-0533
    Keywords: Isolated Nerve Fibres ; Electron Microscopy ; Demyelination
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wird eine Technik zur Isolierung peripherer Nervenfasern durch Auffasern und nachfolgende licht-undd elektronenmikroskopische Untersuchung beschrieben. Diese Technik wurde zum Studium ungewöhnlich geschwollener Fasern angewandt, die proximal der Läsion bei durchschnittenen Nerven von Ratten beobachtet wurden. Diese Fasern wurden als das Ergebnis der Demyelinisation bereits remyelinisierter Segmente dargestellt.
    Notes: Summary A technique is described for isolating peripheral nerve fibres by teasing and subsequently examining them by light and electron microscopy. The technique was applied to the study of unusual swollen fibres observed central to the lesion in transected nerves in rats. These were shown to be the result of the demyelination of already remyelinated segments of the fibre.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 18 (1971), S. 150-159 
    ISSN: 1432-0533
    Keywords: Electron Microscopy ; Nerve Regeneration ; Unmyelinated Axons
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Eine elektronenmikroskopische Untersuchung über die Regeneration von marklosen Axonen wurde am N. vagus des Kaninchens durchgeführt. Als Folge einer lokalisierten Quetschverletzung des N. vagus am Hals in Höhe der Cartilago thyreoidea wurden die regenerierenden marklosen Axone in den laryngealen Recurrens-Ast abgelenkt, der normalerweise beinahe vollständig aus myelinreichen Fasern aufgebaut ist. Die regenerierenden marklosen Axone kommen um die regenerierenden myelinreichen Axone zu liegen, sind aber mit getrennten Schwannschen Zellen verbunden. Eine mögliche Erklärung für diese aberrierende Regeneration der myelinfreien Axone wird diskutiert, ebenso ihre Bedeutung für die Human-Neuropathologie.
    Notes: Summary An electron microscope investigation has been made into the regeneration of unmyelinated axons in the vagus nerve of the rabbit. Following a localized crush injury of the vagus nerve in the neck at the level of the thyroid cartilage, the regenerating unmyelinated axons become diverted into the recurrent laryngeal branch, which is normally composed almost entirely of myelinated fibres. Here the regenerating unmyelinated axons become arrayed around the regenerating myelinated axons, but are associated with separate Schwann cells. The possible explanation for this aberrant regeneration of the unmyelinated axons is discussed, as is its significance for human neuropathology.
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 26 (1973), S. 317-327 
    ISSN: 1432-0533
    Keywords: Peripheral Neuropathy ; Tangier Disease ; Schwann Cells ; Electron Microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Nerve biopsy findings are recorded for two previously reported patients with Tangier disease (hereditary high density lipoprotein deficiency). Both cases showed unusual clinical manifestations in comparison with other reported cases. The neurological disorder, symptoms from which began in the third decade, gave rise to a lower motor neuron deficit of unique distribution, which was accompanied by progressive sensory impairment limited for many years to loss of pain and temperature sensibility, ultimately involving all sensory modalities. Both biopsy specimens displayed similar features, with a gross loss of unmyelinated and myelinated axons, an extensive accumulation of lipid within Schwann cells, and excessive endoneurial collagenization. The axonal loss appeared to represent a primary axonal degeneration, there being no evidence of a demyelinating process. It is suggested that the accumulation of cholesterol within Schwann cells may be the result of a failure of cholesterol removal mechanisms or of intracellular lipid transport.
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Journal of molecular histology 3 (1971), S. 403-404 
    ISSN: 1573-6865
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurocytology 3 (1974), S. 497-512 
    ISSN: 1573-7381
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Observations have been made on the ultrastructural changes that occur in the cervical and abdominal portions of the rabbit vagus nerve following transection. The axonal changes are similar to those that occur during the degeneration of myelinated axons and all axons degenerate completely within 7 days. This time course correlates closely with previous electrophysiological observations. The degeneration of the axons takes place external to the Schwann cells, but these cells tend to react by surrounding the degenerating axons with multiple flattened processes. This debris is removed both by Schwann cells and macrophages. Most of the degenerate material has disappeared by 15 days after transection. Compact columns of Schwann cells analogous to the bands of Büngner are not formed.
    Type of Medium: Electronic Resource
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