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  • 1
    Electronic Resource
    Electronic Resource
    Presence of non-fibrillar amyloid b protein in skin biopsies of Alzheimer's disease (AD), Down's syndrome and non-AD normal persons (1994)
    Springer
    Acta neuropathologica 88 (1994), S. 201-206 
    Details
    ISSN: 1432-0533
    Keywords: Key words Amyloid β protein ; Skin biopsy ; Alzheimer's disease ; Down's syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A total of 66 skin biopsies from persons with Alzheimer's disease (AD) or Down's syndrome (DS) and from persons without AD were used in this study. The age range was from 7 to 89 years. Positive immunoreactivity of skin biopsies to monoclonal antibody 4G8, which is reactive to amino acid residue 17 – 24 of synthetic amyloid β protein (Aβ), and 4G8-Fab (the antigen-binding fragment of 4G8 IgG, reactive only to amyloid plaque) was observed in the epidermis-dermis junction or the basement membrane of the epidermis and in some blood vessels of the biopsy skins of 13/18 (72  %) AD, 9/10 (90  %) DS, and 14/38 (37  %) non-AD control cases. The Fisher exact probability test revealed a significant difference (P=0.0415 one-tailed) in immunoreactivity between AD and age-matched controls. There was also a significant difference (P=0.0152 one-tailed; P=0.0200 two-tailed) between DS and age-matched control in the same test. Immuno-gold electron microscopy examination of these cases with positive immunoreactivity revealed that the gold particles were deposited along the basement membrane of the epidermis. Amyloid fibrils were not observed in the regions with gold particles. Results of this study suggest that Aβ is associated with the basement membrane of skin and is present in amorphous, non-fibrillar form as soluble Aβ.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00293394
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  • 2
    Electronic Resource
    Electronic Resource
    The doublet microtubules of rods of the rabbit retina (1982)
    Springer
    Anatomy and embryology 165 (1982), S. 315-328 
    Details
    ISSN: 1432-0568
    Keywords: Rabbit photoreceptor ; Retina ; Microtubules ; Protofilaments ; Connecting cilium ; Outer doublets ; Microtubule assembly ; Tubulin of oligomers
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The connecting cilium of the rabbit photoreceptor rod is composed of nine outer doublets, lacking dynein side arms. The central singlet microtubules are absent. In cross section, there is an inner dense ring situated between the doublets and the center core of the cilium. As the doublet microtubules progress from the connecting region into outer segments, the cylindrical array of the nine pairs of doublets spreads out as a brush-like arrangement into the incisure cavity of the outer segment. The microtubules continue as doublets for much of the length of the outer segment. The B-tubules terminate first; the A-tubules extend as single tubules into the apical region of the photoreceptor. Before the B-tubules end, they open up, forming hook-shaped projections from the A-tubules. The gradual reduction in length of these hook-shaped structures suggests that near their distal ends each B-tubule opens because of the separation of protofilament 1 of the B-tubule from protofilament 1 of the adjacent A-tubule. Subsequently, the B-tubule protofilaments terminate individually.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00305570
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  • 3
    Electronic Resource
    Electronic Resource
    Purkinje cell dendritic development in experimental phenylketonuria (1981)
    Springer
    Acta neuropathologica 53 (1981), S. 107-112 
    Details
    ISSN: 1432-0533
    Keywords: Dendrites ; Purkinje cells ; Golgi stain ; Experimental phenylketonuria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A comparison was made of cerebellar dendritic development in the normal rat and in a new model of phenylketonuria, the phenylacetate-treated suckling rat. Golgi stain analysis of the Purkinje cells shows striking regional variations in the dendritic growth. These variations were observed in both the control and phenylacetate-treated animals and were especially striking before 15 days of life. Quantitative analysis of the dendritic tree revealed, in the phenylacetate-treated rat, a significant reduction in the total number of dendritic branches. However, the individual terminal dendritic length was largely unaltered. These effects of phenylacetate differ from those of deafferentation and starvation. Results of this investigation clearly define the harmful effects of phenylacetate on developing neurons and are compatible with the clinical observation that brain damage in phenylketonuria occurs mainly during the first few years of life, the critical period of neuronal development.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00689990
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  • 4
    Electronic Resource
    Electronic Resource
    Alzheimer neuropathology in non-Down's syndrome mentally retarded adults (1990)
    Springer
    Acta neuropathologica 80 (1990), S. 362-367 
    Details
    ISSN: 1432-0533
    Keywords: Alzheimer disease ; Mental retardation ; Neuritic plaques ; Neurofibrillary tangles ; Paired helical filaments
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We examined the brains of 385 mentally retarded adults aged 23–90 years without Down's syndrome (DS), metabolic disorder, or hydrocephalus to extend our knowledge about the occurrence of Alzheimer-type neuropathology in this population. Relevant measures of neuropathology also were related to selected information available from clinical records. The presence of one or more neurofibrillary tangles (NFT) and/or neuritic plaques (NP) was observed in 63.4% of all cases and varied with age. The prevalence of positive cases was higher when mental retardation was due to head trauma, congenital malformation, or familial factors and when a history of seizures was reported. Comprehensive morphometric analyses of neocortical, hippocampal and parahippocampal areas indicated that recommended agespecific quantitative criteria for the diagnosis of Alzheimer disease [Khachaturian ZS (1985) Arch Neurol 42:1097–1105] were met in 9.5% of cases less than 50 years of age, 54.2% between 50 and 65, 70% between 66 and 75, and 87% of the cases greater than 75 years of age. However, a limited immunohistochemical study revealed that in most cases the NP did not have a neuritic component containing paired helical filaments and in this respect most of the plaques observed in this population may differ from those most strongly associated with Alzheimer disease. In addition, substantial numbers of NFT were seen in frontal cortex, contrasting with results reported in the literature for nonretarded populations. The number of NP per mm2 consistently increased with age for all areas examined, while the relationship between NFT density and age varied across areas, and was clearly not monotonic. Our studies show that the neuropathological lesions currently considered hallmarks of Alzheimer disease are prevalent among non-DS mentally retarded adults, and the regional density of these lesions is high. Thus, while people with DS are affected at an earlier age, clear Alzheimer neuropathology develops in many mentally retarded individuals.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00307688
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  • 5
    Electronic Resource
    Electronic Resource
    Presence of non-fibrillar amyloid β protein in skin biopsies of Alzheimer's disease (AD), Down's syndrome and non-AD normal persons (1994)
    Springer
    Acta neuropathologica 88 (1994), S. 201-206 
    Details
    ISSN: 1432-0533
    Keywords: Amyloid β protein ; Skin biopsy Alzheimer's disease ; Down's syndrome
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A total of 66 skin biopsies from persons with Alzheimer's disease (AD) or Down's syndrome (DS) and from persons without AD were used in this study. The age range was from 7 to 89 years. Positive immunoreactivity of skin biopsies to monoclonal antibody 4G8, which is reactive to amino acid residue 17–24 of synthetic amyloid β protein (Aβ), and 4G8-Fab (the antigen-binding fragment of 4G8 IgG, reactive only to amyloid plaque) was observed in the epidermis-dermis junction or the basement membrane of the epidermis and in some blood vessels of the biopsy skins of 13/18 (72%) AD, 9/10 (90%) DS, and 14/38 (37%) non-AD control cases. The Fisher exact probability test revealed a significant difference (P=0.0415 one-tailed) in immunoreactivity between AD and age-matched controls. There was also a significant difference (P=0.0152 one-tailed; P=0.0200 two-tailed) between DS and age-matched control in the same test. Immuno-gold electron microscopy examination of these cases with positive immunoreactivity revealed that the gold particles were deposited along the basement membrane of the epidermis. Amyloid fibrils were not observed in the regions with gold particles. Results of this study suggest that Aβ is associated with the basement membrane of skin and is present in amorphous, non-fibrillar form as soluble Aβ.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00293394
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    Paper (German National Licenses)
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  • 6
    Electronic Resource
    Electronic Resource
    Substructures of neurofilaments (1984)
    Springer
    Acta neuropathologica 64 (1984), S. 339-343 
    Details
    ISSN: 1432-0533
    Keywords: Neurofilaments ; Protofilaments ; Substruactures ; Human ; Rabbit
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The results of this investigation indicate that neurofilaments of mammals (human and rabbit) are composed of four protofilaments each of which is formed of globular units connected by longitudinal bars. A cross-view of neurofilaments reveals the presence of four globular units, 20–25 Å in diameter, connected by four transverse bars, 25–30 Å in length. Two of the transverse bars appear more electrondense (darker) than the remaining two. A longitudinal view of neurofilaments shows two protofilaments bonded by the darker of the transverse bars to form a two-stranded band. The remaining two protofilaments form a band in a similar manner. The two two-stranded bands, wrapped helically, form a four-stranded neurofilament. The axial periodicity between twists along each neurofilament ranges from 200 Å to 270 Å. A three-dimensional diagram of neurofilament structure is presented.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00690399
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  • 7
    Electronic Resource
    Electronic Resource
    Experimental phenylketonuria: Effect of phenylacetate intoxication on number of synapses in the cerebellar cortex of the rat (1983)
    Springer
    Acta neuropathologica 61 (1983), S. 313-315 
    Details
    ISSN: 1432-0533
    Keywords: Synaptic density ; Phenylketonuria ; Phenylacetate neurotoxicity
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In experimental phenylketonuria, induced in the rat by exposure to phenylacetate during the first 21 days of life, there was a significant reduction of boutons, a decrease of an average of 25% in the whole cerebellar molecular layer. Both the density of synaptic profiles per square unit and the number of synapses per unit volume were decreased in the phenylacetatetreated rat as compared to the age-matched control. Neuronal density was unaffected. Results are interpreted to show a deficit of synapses per neuron, probably due to a decrease in synaptic formation in phenylacetate-induced phenylketonuria. Undernutrition was eliminated as a contributing factor.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00692004
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  • 8
    Electronic Resource
    Electronic Resource
    Hereditary abnormality in tapetum lucidum of the Siamese cats (1982)
    Springer
    Histochemistry and cell biology 75 (1982), S. 1-9 
    Details
    ISSN: 1432-119X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Gross examination showed a weaker reflection (less shining) of the tapetum lucidum of the Siamese cats compared with common cats. Toluidine blue sections revealed that many tapetal cells were weakly stained and giving vacuolated appearance under high magnification. Further examination with electron microscope showed that those weakly stained cells were filled with disrupted tapetal rods. In these affected cells, the arrangement of the tapetal rods was no longer regular. The membranes of the tapetal rods were either enlarged or disrupted. Some of them appeared to be myelin-like structures. The cores of the tapetal rods were either empty or filled with electron-dense materials which may be the remnant of the original cores. The severity of this type of abnormality or degeneration in the tapetum varied from lavers to layers. Those layers closer to the retina showed a greater number of cells with degeneration. Quantitative analysis of histochemical detection of zinc showed a significantly smaller amount of zinc in tapetal rods of the Siamese cats as compared with common cats. Less zinc and disruption of the regular arrangement of the tapetal rods may result in weaker reflection of light by Siamese cat tapetum. In four of the nine Siamese cats studied, this type of abnormality was observed. It suggests that it is a hereditary disorder of relatively high frequency.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00492528
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  • 9
    Electronic Resource
    Electronic Resource
    Histochemical localization of zinc in the feline tapetum (1981)
    Springer
    Histochemistry and cell biology 72 (1981), S. 341-350 
    Details
    ISSN: 1432-119X
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary High resolution electron microscopy of ultrathin sections confirms the presence of a membrane surrounding the tapetal rods in the cat. Cats depleted of taurine exhibit disruption and disorganization of this membrane, probably the first stage of more severe tapetal degeneration. Histochemical localization of zinc shows it to be present on the periphery of the tapetal rods. The amount of zinc present on the periphery of the tapetal rods of taurine depleted cats was greatly reduced. Taurine in feline tapetum, confirmed by autoradiography and direct measurement, was also greatly reduced in taurine-depleted cats. We conclude that both taurine and zinc are localized on the periphery of the tapetal rods and that they contribute to the stability of the membrane. We have also confirmed earlier reports that the cat tapetal rods contain riboflavin and no detectable cysteine.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00501776
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  • 10
    Electronic Resource
    Electronic Resource
    Aluminum neurotoxicity in mammals (1990)
    Springer
    Environmental geochemistry and health 12 (1990), S. 115-120 
    Details
    ISSN: 1573-2983
    Source: Springer Online Journal Archives 1860-2000
    Topics: Geosciences , Medicine
    Notes: Abstract Although aluminum comprises a large percentage of the Earth's crust, it is excluded from body tissues, and especially from the central nervous system. When aluminum is experimentally introduced to the central nervous system, several neurotoxic effects are observed:i.e. neurofibrillary changes, behavioral and cognitive deficits and enzymatic and neurotransmitter changes, as well as certain types of epileptic seizures. The localization of relatively high levels of aluminum in Alzheimer disease, Guamanian amyotrophic lateral sclerosis and Parkinsonism-dementia has led to the implication of aluminum as a pathogenic factor in these diseases. Recent studies have shown that microtubule-associated proteins are part of the paired helical filaments which make up the intraneuronal neurofibrillary tangle. Other studies have identified the protein making the vascular and neuritic (senile) plaque amyloid and located the gene responsible for this protein to chromosome 21. Our electron microprobe analysis studies have not found the levels of aluminum or silicon in either the neurofibrillary tangles or amyloid cores reported elsewhere, nor have the levels of aluminum been elevated in approximately one half of the tangles and plaque cores examined to date.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01734060
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