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  • 1
    Electronic Resource
    Electronic Resource
    αB-Crystallin is present in reactive glia in Creutzfeldt-Jakob disease (1992)
    Springer
    Acta neuropathologica 83 (1992), S. 324-327 
    Details
    ISSN: 1432-0533
    Keywords: αB-Crystallin ; Immunohistochemistry ; Reactive astrocytes ; Creutzteldt-Jakob disease ; Prion disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary α-Crystallin is a major eye lens protein, composed of two types of subunits, αA and αB. The αA subunit is restricted to the lens, but αB-crystallin has recently also been detected in non-lenticular tissues, including the nervous system. With the use of a polyclonal antiserum directed against a synthetic C-terminal peptide of human αB-crystallin, the presence of αB-crystallin could be demonstrated immunohistochemically in astrocytes in the brains of patients with Creutzfeldt-Jakob disease (CJD). Most intensive localization was observed in the spongiotic tissue representing abundant progressively changed astrocytes in CJD. In agematched control brains weak positive reaction was located in individual oligodendroglia cells and subpial astrocytes. Prominent increase of αB-crystallin in pathological glia in CJD may represent a response to stress.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00296796
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  • 2
    Electronic Resource
    Electronic Resource
    Expression of αB-crystallin in Alzheimer's disease (1994)
    Springer
    Acta neuropathologica 87 (1994), S. 155-160 
    Details
    ISSN: 1432-0533
    Keywords: Key words:αB-crystallin – Heat-shock proteins – Reactive glia – Alzheimer's disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract. αB-crystallin is a member of the small heatshock protein family. Under pathological conditions, the expression of αB-crystallin increases in proliferating astrocytes, which suggests that this protein, in addition to glial fibrillary acidic protein (GFAP), can be a marker for gliosis in neurodegenerative diseases. Immunoblotting and immunohistochemical methods were used for the detection of αB-crystallin in the brains of Alzheimer's disease (AD) patients and nondemented controls. An increase in αB-crystallin expression was found in the brains of AD patients. Immunoreaction was present in reactive astrocytes, microglia, and oligodendrocytes, indicating that all types of glia respond to the stress associated with AD pathology. Colocalization of GFAP and αB-crystallin was found in fibrous astrocytes. However, the intensity and range of αB-crystallin expression appeared to be limited as compared with the large increase in the number of GFAP-positive astrocytes. This indicates that expression of αB-crystallin is not a marker for gliosis in AD. Immunoreactivity to αB-crystallin in both astrocytes and microglia was found mainly restricted to areas with senile plaques and neurofibrillary tangles, suggesting the association of αB-crystallin with amyloid deposition AD.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00296185
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Congenital muscular dystrophy and severe central nervous system atrophy in two siblings (1995)
    Springer
    Acta neuropathologica 90 (1995), S. 650-656 
    Details
    ISSN: 1432-0533
    Keywords: Key words Congenital muscular dystrophy ; Cerebral atrophy ; Myelin deficiency ; Multisystem degeneration ; Sural nerve
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Severe degenerative features of the nervous system of a hitherto unknown kind, associated with a neuromuscular disorder with histopathological features of congenital muscular dystrophy, are reported in two female siblings. The clinical profile was characterized by generalized hypotonia followed by spastic tetraplegia, contractures, polyneuropathy, lack of cognitive development and progressive microcephaly. There was no involvement of the eyes. Neuropathological examination of the brain of one sibling, who died at the age of 30 months, revealed subtotal loss of neurons in the cerebral and cerebellar cortex and in the ventral pons, and secondary loss of myelin in the cerebral and cerebellar subcortical white matter. Sural nerve biopsy in the other sibling, who had a similar neurological affection, showed a lack of large myelinated fibers.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00318580
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  • 4
    Electronic Resource
    Electronic Resource
    Neuropathological findings in muscle-eye-brain disease (MEB-D) (1991)
    Springer
    Acta neuropathologica 83 (1991), S. 55-60 
    Details
    ISSN: 1432-0533
    Keywords: Muscle-eye-brain disease ; Fukuyama type of congenital muscular dystrophy ; Neuropathological differentiation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Congenital muscular dystrophy (CMD) associated with cerebro-ocular dysplasia named muscle-eye-brain disease (MEB-D) is described in two sisters. Progressive hypotonia, mental retardation and severe visual failure appeared immediately after birth. Pathological examination demonstrated muscular dystrophy, hydrocephalus, type II lissencephaly and defective eye development of foetal origin. The great similarity of the clinical and neuropathological picture of both sisters is in agreement with an autosomal recessive inheritance. Neuropathological distinction between Fukuyama-CMD and MEB-D is a more severe and earlier cerebral developmental defect and the association with ocular dysplasia in MEB-D.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294430
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    Paper (German National Licenses)
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  • 5
    Electronic Resource
    Electronic Resource
    Expression of αB-crystallin in Alzheimer's disease (1994)
    Springer
    Acta neuropathologica 87 (1994), S. 155-160 
    Details
    ISSN: 1432-0533
    Keywords: αB-crystallin ; Heat-shock proteins ; Reactive glia ; Alzheimer's disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract αB-crystallin is a member of the small heatshock protein family. Under pathological conditions, the expression of αB-crystallin increases in proliferating astrocytes, which suggests that this protein, in addition to glial fibrillary acidic protein (GFAP), can be a marker for gliosis in neurodegenerative diseases. Immunoblotting and immunohistochemical methods were used for the detection of αB-crystallin in the brains of Alzheimer's disease (AD) patients and nondemented controls. An increase in αB-cyrstallin expression was found in the brains of AD patients. Immunoreaction was present in reactive astrocytes, microglia, and oligodendrocytes, indicating that all types of glia respond to the stress associated with AD pathology. Colocalization of GFAP and αB-crystallin was found in fibrous astrocytes. However, the intensity and range of αB-crystallin expression appeared to be limited as compared with the large increase in the number of GFAP-positive astrocytes. This indicates that expression of αB-crystallin is not a marker for gliosis in AD. Immunoreactivity to αB-crystallin in both astrocytes and microglia was found mainly restricted to areas with senile plaques and neurofibrillary tangles, suggesting the association of αB-crystallin with amyloid deposition in AD.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00296185
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    Paper (German National Licenses)
    Fulltext
  • 6
    Electronic Resource
    Electronic Resource
    Expression of small heat-shock protein hsp 27 in reactive gliosis in Alzheimer disease and other types of dementia (1994)
    Springer
    Acta neuropathologica 87 (1994), S. 511-519 
    Details
    ISSN: 1432-0533
    Keywords: Alzheimer disease ; Dementia ; Stress-response proteins ; small heat-shock protein 27 ; Reactive astrocytes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Immunohistochemical and immunoblotting analysis of brain tissue of Alzheimer's disease (AD) patients showed highly induced expression of the small heat-shock protein hsp 27 in affected cortex. Expression of hsp 27 was present in a large number of proliferating astrocytes. The highest expression was exhibited by degenerative astrocytes in the areas rich in senile plaques. Neurofibrillary tangles, Hirano bodies and some hippocampal neurons were also positive. Expression of hsp 27 increased with the severity of AD-specific morphological changes, and with the duration of dementia. In control brains immunoreaction was restricted to the vessels and to occasional astrocytes in the white matter. Similar patterns of immunoreactivity were present in cases without dementia (Parkinson disease, lacunar state, or focal ischemic necrosis). Patients suffering from other types of dementia (Parkinson/dementia complex, multi-infarct dementia, normal pressure hydrocephalus) showed less expression of hsp 27 in reactive astrocytes than AD, but more than controls. These results indicate that increased expression of hsp 27, especially in astrocytes showing klazmatodendrosis, is associated with AD pathology.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294178
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  • 7
    Electronic Resource
    Electronic Resource
    Proteins immunologically related to erythrocyte anion transporter band 3 are altered in brain areas affected by Alzheimer's disease (1993)
    Springer
    Acta neuropathologica 86 (1993), S. 353-359 
    Details
    ISSN: 1432-0533
    Keywords: Aging ; Alzheimer's disease ; Anion exchange ; Band 3 ; Immunochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Proteins immunologically related to the human erythrocyte anion transporter band 3 are present in neurons of the human neocortex and hippocampus. Immunocytochemical studies show increased band 3 immunoreactivity in neurons in the brains of patients with Alzheimer's disease. Immunoblot studies show the presence of band 3-like molecules in brain membrane fractions, and suggest changes in expression and/or processing of band 3-like molecules in Alzheimer's disease-affected regions. We postulate that alterations in membrane-bound, band 3-like molecules may reflect termination of neuronal lifespan in Alzheimer's disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00369447
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    Paper (German National Licenses)
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  • 8
    Electronic Resource
    Electronic Resource
    Congenital muscular dystrophy and severe central nervous system atrophy in two siblings (1995)
    Springer
    Acta neuropathologica 90 (1995), S. 650-656 
    Details
    ISSN: 1432-0533
    Keywords: Congenital muscular dystrophy ; Cerebral atrophy ; Myelin deficiency ; Multisystem degeneration ; Sural nerve
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Severe degenerative features of the nervous system of a hitherto unknown kind, associated with a neuromuscular disorder with histopathological features of congenital muscular dystrophy, are reported in two female siblings. The clinical profile was characterized by generalized hypotonia followed by spastic tetraplegia, contractures, polyneuropathy, lack of cognitive development and progressive microcephaly. There was no involvement of the eyes. Neuropathological examination of the brain of one sibling, who died at the age of 30 months, revealed subtotal loss of neurons in the cerebral and cerebellar cortex and in the ventral pons, and secondary loss of myelin in the cerebral and cerebellar subcortical white matter. Sural nerve biopsy in the other sibling, who had a similar neurological affection, showed a lack of large myelinated fibers.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00318580
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
  • 9
    Electronic Resource
    Electronic Resource
    Galanin-like immunoreactivity within Ch2 neurons in the vertical limb of the diagonal band of Broca in aging and Alzheimer's disease (1989)
    Springer
    Acta neuropathologica 78 (1989), S. 90-95 
    Details
    ISSN: 1432-0533
    Keywords: Galanin ; Immunochemistry ; Nucleus basalis of Meynert complex ; Hippocampus ; Alzheimer's disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The neuropeptide galanin is known to inhibit the evoked release of acetylcholine in ventral hippocampus of the rat. Co-localization of this peptide with choline acetyltransferase in neurons of the cholinergic septal nuclei has been demonstrated in the rat and non-human primate. The severe deficiency of the cholinergic hippocampal projection system arising mainly from the vertical limb nucleus of the diagonal band of Broca, also referred to as Ch2 region, is a constant finding in Alzheimer's disease, a disorder which is neuropathologically characterized by the appearance of senile plaques, neurofibrillary tangles and congophilic angiopathy in neo- and archicortical structures. In the present study for the first time galanin immunoreactivity in the human Ch2 region is morphologically investigated and related to the severity of hippocampal plaques and neurofibrillary tangles in Alzheimer's disease. An inverse relationship between decreasing galanin immunoreactivity in the Ch2 region and amounts of senile plaques and neurofibrillary tangles in the hippocampus is indicated. Considering the cholinergic deficiency in Alzheimer's disease as a secondary phenomenon to primary cortical and hippocampal lesions, and realizing the inhibitory effect of galanin upon acetylcholine release in hippocampus, this preliminary study suggests that a decreased galanin immunoreactivity in Ch2 in Alzheimer's disease reflects a possible negative feedback mechanism to a degenerating cholinergic projection system.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00687407
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    Paper (German National Licenses)
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  • 10
    Electronic Resource
    Electronic Resource
    Congenital muscular dystrophy (1993)
    Springer
    Acta neuropathologica 86 (1993), S. 386-392 
    Details
    ISSN: 1432-0533
    Keywords: Congenital muscular dystrophy (CMD) ; Muscle biopsy ; Semiquantitative histological analysis ; Dystrophin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Histomorphological and histochemical variability was studied in muscle specimens from 30 patients with congenital muscular dystrophy (CMD). We found involvement of the central nervous system in 8 patients (Fukuyama CMD, F-CMD), involvement of the brain and the eyes in 5 patients (muscle, eye and brain disease, MEB-D) and hypodense white matter on the CT scans of 2 patients with (sub)normal intelligence (occidentaltype cerebromuscular dystrophy, O-CMD). No morphological hallmarks were found to differentiate these subgroups. Only fat cell infiltration was found to be increased with increasing age in ‘pure’ CMD (pure-CMD). The morphological data did not appear to be correlated with the clinical severity or type of dystrophy (pure-CMD, F-CMD, MEB-D and O-CMD). Immunohistochemistry with dystrophin, vimentin and desmin antibodies in 14 patients (6 pure-CMD, 5 F-CMD, 2 MEB-D and 1 O-CMD) showed a normal expression pattern.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00369452
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    Paper (German National Licenses)
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