| 101. |
- Tågerud, Sven, et al.
(author)
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An ultrastructural study of the segmental uptake of horseradish peroxidase in the endplate region of denervated skeletal muscle fibres
- 1986
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In: Journal of the Neurological Sciences. - 0022-510X .- 1878-5883. ; 75:2, s. 141-157
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Journal article (peer-reviewed)abstract
- The segmental uptake of horseradish peroxidase (HRP) in the endplate region of denervated skeletal muscle fibres has been studied ultrastructurally using a method for selecting single muscle fibres with high segmental peroxidase staining from denervated mouse tibialis anterior muscle.Segments containing large peroxidase positive phagosomes could already be seen 10–15 min after i.v. injection of HRP. Such segments were still present 24 h after HRP injection. The localization of phagosomes, deep in the fibres rather than immediately under the sarcolemma, suggests that the uptake occurs from t-tubuli. Vivid proliferation of t-tubuli, consisting of vesiculation, enlargement and encircling of cytoplasmic components, was also observed. The HRP accumulates in phagosomes of varying size and shape. Similar membrane-limited bodies without or with very weak peroxidase staining were also observed. The peroxidase-positive phagosomes participate in autophagic processes as suggested by their content of undegraded cellular material. Golgi profiles, which occurred deep in the muscle fibres, and enlarged components of the sarcoplasmic reticulum were frequently encountered in the segments. Myofibrillar degeneration occurs in the segments and progresses with time after denervation.The described segments may be related to the increased membrane turnover in denervated muscle fibres and/or they may be related to processes aimed at establishing new synaptic contacts.
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| 102. |
- Tågerud, Sven, et al.
(author)
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Biochemical and ultrastructural effects of chloroquine on horseradish peroxidase uptake and lysosomal enzyme activities in innervated and denervated mouse skeletal muscle
- 1986
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In: Journal of the Neurological Sciences. - 0022-510X .- 1878-5883. ; 75:2, s. 159-171
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Journal article (peer-reviewed)abstract
- The effects of chloroquine treatment on horseradish peroxidase (HRP) uptake and lysosomal enzyme activities in innervated and denervated mouse skeletal muscle have been studied using biochemical, histochemical and ultrastructural techniques.Chloroquine treatment caused a large (59–101%) increase in the activity of cathepsin D in both innervated and denervated muscle. The activity of N-acetyl-β-d-glucosaminidase also increased slightly in denervated muscle. No effect was observed on acid phosphatase activity. The in vivo uptake of HRP in innervated and denervated muscle was unaffected by chloroquine treatment. The results show that the activities of certain lysosomal enzymes may increase in skeletal muscle without an increase in endocytic activity. This is discussed in comparison to what is seen in denervated and dystrophic muscle.Histochemical and ultrastructural studies showed the HRP uptake to occur segmentally in denervated muscle fibres from untreated as well as chloroquine-treated animals. Ultrastructurally the peroxidase-positive phagosomes occurring in these segments were found to contain increased levels of undegraded material after chloroquine treatment suggesting that these phagosomes are of a lysosomal nature and also participate in autophagic processes.
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| 103. |
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| 104. |
- Verkkoniemi, Auli, et al.
(author)
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Neuropsychological functions in variant Alzheimer's disease with spastic paraparesis
- 2004
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In: Journal of the Neurological Sciences. - : Elsevier BV. - 0022-510X .- 1878-5883. ; 218:1-2, s. 29-37
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Journal article (peer-reviewed)abstract
- Few data exist on the effects of specific Alzheimer's disease (AD)-related mutations on cognitive function. We present neuropsychological test results in eight members of a large kindred with variant Alzheimer's disease (VarAD) due to a deletion of the presenilin 1 (PS-1) gene, encompassing exon 9. The disease was neuropathologically characterized by the presence of large, unusual, "cotton wool" plaques (CWP). Four surviving patients were prospectively tested, and retrospective neuropsychological data were collected from additional four deceased patients. The neuropsychological evaluation was based on tests of verbal and visual memory, abstract thinking, and visuoconstructive and spatial functions. In addition, psychiatric symptoms were evaluated. In four patients, brain glucose metabolism was examined by positron emission tomography (PET). PET showed temporoparietal hypometabolism typical of AD. In addition, variable patterns of hypometabolism (hemispherical asymmetry and occipital accentuation) were related to individual deficits of cognitive performance. However, all these early-onset patients (age range 43-63 years) with a deletion mutation of PS-1 gene showed prominent memory impairment and deficits in visuoconstructive and intellectual functions.
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| 105. |
- Vrethem, Magnus, et al.
(author)
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A patient with both narcolepsy and multiple sclerosis in association with Pandemrix vaccination
- 2012
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In: Journal of the Neurological Sciences. - : Elsevier BV. - 0022-510X .- 1878-5883. ; 321:1-2, s. 89-91
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Journal article (peer-reviewed)abstract
- Narcolepsy with cataplexy is caused by a selective loss of hypocretin-producing neurons, but symptomatic narcolepsy can also result from hypothalamic and brainstem lesions caused by multiple sclerosis (MS). We report a previously healthy man who developed clinical and laboratory verified narcolepsy without having any indication of hypothalamic lesions and MS after vaccination against the influenza H1N1 with Pandemrix. HLA typing showed both DRB1*15:01, associated with MS and DQB1*06:02, associated with narcolepsy. The genetic susceptibility in this patient makes it tempting to speculate upon an immune-mediated mechanism and a common etiology for both diseases in this patient. (C) 2012 Elsevier B.V. All rights reserved.
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| 106. |
- Vult von Steyern, Fredrik, et al.
(author)
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Endocytotic activity of mouse skeletal muscle fibres after long-term denervation
- 1994
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In: Journal of the Neurological Sciences. - 1878-5883. ; 125:2, s. 147-152
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Journal article (peer-reviewed)abstract
- The endocytotic activity of skeletal muscle fibres and its relation to the denervated endplate region has been studied using horseradish peroxidase (HRP) as marker for endocytosis. In muscles denervated for a short time period (10-20 days) HRP-uptake occurred in small segments of the muscle fibres near the centre of the muscle (endplate region). After long-term denervation (6-12 months) similar segments with high endocytotic activity were seen preferentially in more peripheral parts of the muscle fibres. Ultrastructural characteristics of segments with high endocytotic activity from long-term denervated muscle fibres include a proliferating transverse tubular system, HRP-containing bodies of different sizes with some very large vacuoles extending over several sarcomeres. These characteristics are similar to those described previously for HRP-uptake in the endplate region of short-term denervated muscle (Tagerud et al., J. Neurol. Sci., 75 (1986) 141) except that no recognizable endplate structures were observed in the present study. The results are discussed in relation to the fate of the denervated endplate and the receptive capacity for synapse formation in long-term denervated muscle.
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| 107. |
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| 108. |
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| 109. |
- Zachau, A C, et al.
(author)
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Distribution of plasma cells secreting antibodies against nervous tissue antigens during experimental allergic encephalomyelitis enumerated by a nitrocellulose immunospot assay.
- 1989
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In: Journal of the Neurological Sciences. - 0022-510X .- 1878-5883. ; 91:3, s. 323-336
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Journal article (peer-reviewed)abstract
- The B cell response to central nervous system (CNS) myelin and myelin basic protein, as well as total numbers of IgG secreting cells, was studied in acute experimental allergic encephalomyelitis using a nitrocellulose immunospot assay. The method was able to detect single plasma cells secreting antibodies. Cells secreting antibodies against myelin antigens were detected in regional lymph node cell suspension by day 5 post-immunization (p.i.). At that time no anti-myelin antibodies were detected free in serum. Later, at day 15 p.i., specific antibody secreting cells were found in bone marrow and spleen indicating a generalization of the immune response. The B cell response became partly sequestered to the target of immune attack since an increased number of IgG secreting cells was detected among mononuclear cells recovered from the CNS. Studies of cellular secretion of antibodies rather than free levels in body fluids may be a more accurate reflection of the in vivo B cell response. These findings may be generally considered in studies of B cell mediated immunity in neuroinflammatory diseases.
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| 110. |
- Zetterberg, Henrik, 1973, et al.
(author)
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Association of APOE with age at onset of sporadic amyotrophic lateral sclerosis.
- 2008
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In: Journal of the neurological sciences. - : Elsevier BV. - 0022-510X .- 1878-5883. ; 273:1-2, s. 67-9
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Journal article (peer-reviewed)abstract
- Amyotrophic lateral sclerosis (ALS) is a neurodegenerative syndrome with familial and sporadic forms. We conducted a study including 60 sporadic and 19 familial ALS patients, 206 reference patients with other neurological disorders and 1265 neurologically healthy controls to assess the Alzheimer-associated apolipoprotein E (APOE) epsilon4 gene variant as a possible risk factor for ALS. While no major influence of APOE epsilon4 on disease risk was detected, a gene dose-dependent effect with lower age at onset of sporadic ALS in epsilon4 carriers was found (p=0.027). These data support APOE epsilon4 as a subordinate contributing factor in ALS.
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