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  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Histopathology 21 (1992), S. 0 
    ISSN: 1365-2559
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Eleven colloid cysts of the third ventricle were investigated by immunohistochemistry using 16 mono- and polyclonal antibodies. Colloid cyst epithelium, choroid plexus epithelium and ependyma showed different immunohistochemical profiles. In particular, antibodies to different cytokeratins and to transthyretin revealed considerable and constant differences of immunoreactivity. Therefore, common derivation of the three tissues seems unlikely. Our findings are interpreted in favour of the non-neuroepithelial origin of third ventricular colloid cysts.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1460-9568
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: The closely related synaptic vesicle membrane proteins synaptophysin and synaptoporin are abundant in the hippocampal formation of the adult rat. But the prenatal hippocampal formation contains only synaptophysin, which is first detected at embryonic day 17 (E17) in perikarya and axons of the pyramidal neurons. At E21 synaptophysin immunoreactivity extends into the apical dendrites of these cells and in newly formed terminals contacting these dendrites. The transient presence of synaptophysin in axons and dendrites suggests a functional involvement of synaptophysin in fibre outgrowth of developing pyramidal neurons. Synaptoporin expression parallels the formation of dentate granule cell synaptic contacts with pyramidal neurons: the amount of hippocampal synaptoporin, determined in immunoblots and by synaptoporin immunostaining of developing mossy fibre terminals, increases during the first postnatal week. Moreover, in the adult, synaptoporin is found exclusively in the mossy fibre terminals present in the hilar region of the dentate gyrus and the regio inferior of the cornu ammonis. In contrast, synaptophysin is present in all synaptic fields of the hippocampal formation, including the mossy fibre terminals, where it colocalizes with synaptoporin in the same boutons. Our data indicate that granule neuron terminals differ from all other terminals of the hippocampal formation by the presence of both synaptoporin and synaptophysin. This difference, observed in the earliest synaptic contacts in the postnatal hippocampus and persisting into adult life, suggests distinct functions of synaptoporin in these nerve terminals.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1439-0264
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Carcinoma-in-situ (CIS) of the testis is known to be the pre-invasive stage of most human germ cell tumours (seminoma and non-seminoma), but the mechanisms leading to an increased pubertal proliferation of CIS cells after a long latency and to progression of CIS to an invasive malignancy are still not known. Additionally, CIS and seminoma have also been reported in equine testis (Veeramachaneni and Sawyer, 1998). The gap junctional protein and tumour suppressor gene connexin(cx)43 represents the predominant cx in human, canine and rodent testis so far and it is expected to play a key role for the regulation of both proliferation and differentiation of germ cells (spermatogonia and spermatocytes), and its gene- and protein-expression pattern is typical for the pubertal terminal differentiation of somatic Sertoli cells. Using cDNA-microarray analysis, in-situ hybridization (ISH), RT-PCR from tissue homogenate and semi-quantitative RT-PCR from well defined microdissected tubules with normal spermatogenesis, CIS, intratubular seminoma (ISe) and from seminoma cells from invasive seminoma we found a downregulation of cx43 starting in intratubular CIS, leading to a complete loss in most invasive seminoma cells. This indicates that regulation of cx43 expression takes place at transcriptional level confirming and expanding earlier studies of protein expression (Brehm et al., 2002). This reduction of cx43-expression suggests that an early intratubular derangement in cx43-gene expression and disruption of inter-cellular communication between Sertoli cells and/or Sertoli cells and pre-invasive tumour cells via cx43-gap junctions may play a role in the proliferation of CIS cells and seminoma cells and in the progression phase of testicular seminoma development.References  Veeramachaneni, D. N., and H. R.Sawyer, 1998: Carcinoma in situ and seminoma in equine testis. APMIS 106, 183–185.Brehm R., A. Marks, R. Rey, S. Kliesch, M. Bergmann and K. Steger, 2002: Altered expression of connexins 26 and 43 in Sertoli cells in seminiferous tubules infiltrated with carcinoma-in-situ or seminoma. J. Pathol. 197, 647–653.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Berlin, Germany : Blackwell Verlag GmbH
    Anatomia, histologia, embryologia 34 (2005), S. 0 
    ISSN: 1439-0264
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Efficient and well-regulated intercellular communication is essential to organize the activities of the different cell populations involved during growth and concomitant structural maturation of the skeleton. For direct cell-cell communication gap junctions composed of connexins (Cx) are present. So far 21 Cx types have been identified in mammals, which differ in molecular permeability as well as tissue expression pattern. To characterize the Cx distribution in the developing canine skeleton, samples from humerus, scapula and lumbar vertebrae of Beagle dogs aged 1.5, 3, 4.5 months and 1.5 years were fixed in Bouin's solution, decalcified with 25% EDTA and embedded in paraffin wax. Using immunohistochemistry and in situ hybridization, the expression of Cx 26, 30, 31, 32, 43, and 45 was studied in bone tissue plus associated epiphyseal and growth plate cartilage. Of all bone cells, osteoblasts showed the largest Cx expression repertoire: they were positive for Cx 26, 30, 32 and 43. After transformation into bone lining cells Cx43 expression was lost while in osteocytes only Cx 26 and 32 was detected. A wide variety of Cx reactions was also found in osteoclasts: Cx 26, 30, 32, and 43. In contrast, chondrocytes showed a much more restricted Cx expression pattern. Positive reactions were only seen in hypertrophic chondrocytes of epiphyseal cartilage (Cx 26, 31, 32) and in the proliferative (Cx32) and hypertrophic zone (Cx 26 and 32) of growth plate cartilage. Additionally, Cx31 was strongly expressed in the cellular layer of the periosteum covering areas of intensive bone modelling in the puppies. Age- and microlocation-dependent variations in expression and/or staining intensity were also observed in the other Cx types. These results demonstrated that canine bone cells are capable of synthesizing a wide variety of Cx types during skeletal development, thus propagating different cellular signals via gap junctions in a differentially regulated and requirement orientated manner.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1433-0350
    Keywords: Neonatal intracranial aneurysm ; Cerebral aneurysm in childhood ; Familial intracranial aneurysm ; Collagen type III ; Cerebral hemorrhage
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 32-day-old boy died of recurring cerebral hemorrhages starting on the 4th day of life. Autopsy disclosed a remittingly ruptured saccular aneurysm of the anterior communicating artery. A 7-day-old brother of his had previously died of recurring subarachnoid hemorrhages as well. The young age of the patient, the site of the aneurysm, and its probably familial occurrence make this case a unique one. Nonaneurysmatic basal cerebral arteries showed remarkable histological changes partly resembling those seen in fibromuscular dysplasia, some of them probably representing preaneurysmatic alterations. A known underlying systemic disease could not be found, and immunohistochemical detection of type III collagen revealed no identifiable deficiency.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 9 (1993), S. 394-399 
    ISSN: 1433-0350
    Keywords: Lissencephaly ; Agyria ; Neuronal migration ; Miller-Dieker syndrome ; Walker-Warburg syndrome ; Congenital muscular dystrophy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The neuropathological findings at autopsy in four cases of type I and three of type II lissencephaly are presented. Type I lissencephaly is characterized by agyriapachygyria with a markedly thickened cerebral cortex with four coarse histological layers. The normally myelinated white matter, often with neuronal heterotopias, is very narrow, and the gray-to-white matter ratio is inverted (about 4:1); there are no white-gray interdigitations. Claustrum and capsula extrema are absent. Ventricular dilatation is present, especially of the occipital horns. In the hypoplastic brain stem large olivary heterotopias can often be observed. Severe cerebellar malformations, obstructive hydrocephalus, severe eye abnormalities, and congenital muscular dystrophy are not seen. Clinically, type I lissencephaly presents as “isolated lissencephaly sequence” or as “Miller-Dieker syndrome” with characteristic facial dysmorphism. The long survival of 20 years achieved by one of our patients is very uncommon. Type II lissencephaly is characterized by widespread agyria. Usually, obstructive hydrocephalus is present with a thin cerebral mantle showing a slightly thickened cortex and a narrow, hypomyelinated white matter often with neuronal heterotopias (gray-to-white matter ratio about 1:1). The border between gray and white matter is blurred. Claustrum and capsula extrema are absent. Histologically, the cortex appears disorganized without layering; widespread leptomeningeal gliomesenchymal proliferations and glioneuronal heterotopias are present. Hydrocephalus is caused by obstruction of the subarachnoid space, aqueductal stenosis, and Dandy-Walker malformation. The latter is a typical finding in type II lissencephaly together with severe cerebellar cortical dysplasias. Aplasia of the olfactory nerves, gliotic fusion of the frontal lobes, and an occipital cephalocele may be found. Type II lissencephaly occurs in clinical syndromes with severe eye abnormalities and congenital muscular dystrophy (the so-called “Walker-Warburg syndrome”). The congenital muscular dystrophies have no dystrophin deficiency, as we were able to show immunohistochemically in the muscle biopsies of two further patients with this syndrome.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 86 (1993), S. 105-108 
    ISSN: 1432-0533
    Keywords: Pallido-luysio-nigral atrophy ; Motor neuron disease ; Ubiquitin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of motor neuron disease (MND) with pallido-luysio-nigral atrophy (PLNA) is reported. The 45-year-old male patient presented with lower motor neuron symptoms and signs of basal ganglia disturbance. He died after a progressive course of 7 months. Neuropathological examination revealed motor neuron loss at all spinal cord levels with sparing of Onuf's nucleus. Nerve cell loss and gliosis were also present in substantia nigra, globus pallidus, and subthalamic nucleus. The presence of ubiquitin-positive inclusions, a hallmark of most variants of MND, confirms this case as an example of MND. At immunoelectron microscopy the granules were distributed on filamentous material. The combination of clinically apparent PLNA with MND has only been described twice previously. The relationship of this syndrome to other forms of MND and its nosological placement are discussed.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-0533
    Keywords: Key words Pineal parenchymal tumors ; Pineocytoma ; Pineoblastoma ; Central neurocytoma ; Neuronal differentiation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Two pineal parenchymal tumors are presented, arising in a 54-year-old man and a 72-year-old woman; respectively. They showed isomorphic, cellular areas of small cells, often with characteristic pineocytomatous rosettes, and of medium-sized cells, as well as less cellular regions with highly pleomorphic, often ganglioid large cells. Immunohistochemistry disclosed extensive neuronal differentiation. There was intense positivity for neurofilament protein and microtubule-associated protein 2 in the pleomorphic areas and more variable expression in the isomorphic regions. Diffuse synaptophysin positivity was seen, accentuated along the borders of pleomorphic cells and in the rosettes, as well as diffuse interstitial and/or cytoplasmic expression of neuron-specific enolase, PGP 9.5 and tau. β-Tubulin III was detected in most cells and slight positivity was found in the rosettes. Expression of glial fibrillary acidic protein, however, was restricted to resident astrocytes and an interstitial network of processes. These neuronally differentiated pleomorphic pineocytomas underline the broad histomorphological spectrum of pineal parenchymal tumors.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-0533
    Keywords: Infantile multiple system atrophy ; Ubiquitin ; Neuronal intraunclear hyaline inclusion disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report presents a case of infantile multiple system atrophy with probably autosomal recessive inheritance. The female patient developed generalized muscular hypotonia, myoclonias and tonic-clonic seizures at the age of 8 months, followed by gradual development of choreoathetotic hyperkinesia and increasing psychomotor retardation. Metabolic disease was ruled out and the child died of aspiration pneumonia at the age of 5 years. General autopsy was unremarkable, but neuropathological examination showed degeneration of cerebellum, inferior olives, medial thalamus, Clarke's nucleus, anterior horn cells, corticospinal, spinocerebellar tracts, and posterior columns. Immunohistochemically many neurons contained intranuclear and intracytoplasmic ubiquitin-positive inclusions, which did not contain neurofilament or tau epitopes and ultrastructurally consisted of granulofilamentous material. We tentatively classify this case as a form of infantile multiple system atrophy linked to neuronal intranuclear hyaline inclusion disease.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-0533
    Keywords: KeyWordsInfantile multiple system atrophy Ubiquitin ; Neuronal intranuclear hyaline inclusion disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report presents a case of infantile multiple system atrophy with probably autosomal recessive inheritance. The female patient developed generalized muscular hypotonia, myoclonias and tonic-clonic seizures at the age of 8 months, followed by gradual development of choreoathetotic hyperkinesia and increasing psychomotor retardation. Metabolic disease was ruled out and the child died of aspiration pneumonia at the age of 5 years. General autopsy was unremarkable, but neuropathological examination showed degeneration of cerebellum, inferior olives, medial thalamus, Clarke's nucleus, anterior horn cells, corticospinal, spinocerebellar tracts, and posterior columns. Immunohistochemically many neurons contained intranuclear and intracytoplasmic ubiquitin-positive inclusions, which did not contain neurofilament or tau epitopes and ultrastructurally consisted of granulofilamentous material. We tentatively classify this case as a form of infantile multiple system atrophy linked to neuronal intranuclear hyaline inclusion disease.
    Type of Medium: Electronic Resource
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