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  • 1
    ISSN: 1432-0533
    Keywords: Neuronal ceroid lipofuscinosis ; Batten disease ; Lipopigment ; Storage disease ; Amyloid precursor protein
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary To verify our hypothesis of defective protease inhibitor domains that are encoded by abnormal processing of amyloid precursor protein (APP) in brains of patients with neuronal ceroid lipofuscinoses (NCL), immunohistochemical and cytochemical studies were performed with monoclonal antibodies (mAbs) directed against various domains of APP. For the studies, 22 autopsy brains were used: 12 with different forms of NCL, and 10 control brains. The staining procedure for the avidin-biotin complex (ABC) technique and the postembedding gold-labelled procedure for electron microscopy (EM) were employed. Of all mAbs used for the study, only mAbs generated against amyloid B-protein bound to neural tissue were affected with NCL. The strongest immunostaining of neurons and of some reactive glial cells was found in brains with the juvenile form of NCL. Only in the infantile form of the disease were some neurons overloaded with storage material weakly immunoreactive. In brains of patients with the adult form of NCL, immunoreactivity was found in affected neurons and in extracellularly deposited material of senile plaques. The results of EM study showed that the immunoreactivity was restricted to lysosomal cytosomes in neural tissue with any form of NCL selectively localized on the curvilinear and fingerprint proteinaceous component of ceroid lipofuscin. Studies performed on control aging brains and Alzheimer's disease (AD) brains confirmed previous observations of immunoreactivity being found diffusely in the protein component of some neurons containing lipopigment. The defective processing of APP in brains with NCL and AD and in ageing brains is discussed. Our present results support the notion of heterogeneity of ceroid lipofuscin storage material in various forms of NCL and underline the hypothesis that abnormalities found in the protease inhibitors or APP in the proteinaceous composition of storage lipopigment could be a key to the unknown etiology of NCL.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1459
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1459
    Keywords: Myasthenic syndrome ; Myasthenic myopathy ; Neuromuscular junction
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Two siblings with congenital myopathy showing myasthenic manifestations together with congenital cataract are reported. Their muscle weakness fluctuated and was alleviated by edrophonium chloride. Their serum creatine kinase activity was elevated, and the waning phenomenon was observed on repetitive nerve stimulation. Biopsied muscle showed degenerative changes with type 1 fibre predominance and abnormal morphology of neuromuscular junctions.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1573-0603
    Keywords: neurons ; primary culture ; collagen ; fibronectin ; laminin ; heparan sulfate proteoglycan
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology
    Notes: Summary We used components of the extracellular matrix (ECM), which plays an important role in neuronal development, as coating materials for culture dishes. Components used were fibronectin (FN), laminin (LN), heparan sulfate proteoglycan (HSPG), type I collagen (COL-I), type IV collagen (COL-IV), and type VIII collagen (COL-VIII). We obtained highly neuron-enriched primary cultures from embryonic mouse whole brain by careful handling of materials, improvement of serum-free medium components, and reduction of seeding cell density. Neuronal cells were well differentiated with ECM (FN: 63.8%, LN: 76.2%, HSPG: 75.3%, COL-I: 63.3%, COL-IV: 55.4%, COL-VIII: 72.2%, respectively, on Day 5 in culture as assessed by determining percentages of neurite-bearing cells), whereas glial fibrillary acidic protein-positive cells remained minimized (FN: 3.6%, LN: 3.2%, HSPG: 2.8%, COL-I: 2.1%, COL-IV: 3.1%, respectively, on Day 5 in culture).
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1955
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract  Mice that have recovered from a primary infection with Plasmodium chabaudi have been shown to resist a secondary infection. In the present study the authors investigated how natural killer (NK) cells were involved in this resistance. Spleen cells from P. chabaudi-primed C57BL/6 mice could transfer protection against P. chabaudi infection into naive syngeneic mice, but spleen cells from unprimed mice could not. T-enriched cells purified from primed spleen cells could also transfer such protection. Transfer of NK cells from primed spleen cells failed to protect against challenge infection. However, depletion of NK cells in host mice by injection of an anti-NK1.1 monoclonal antibody resulted in higher mortality relative to controls. The possible protective roles of NK cells in P. chabaudi infection are discussed.
    Type of Medium: Electronic Resource
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