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  • 1
    ISSN: 1432-0533
    Keywords: Key words Motor neuron disease ; Vertical gaze palsy ; Progressive supranuclear palsy ; Multiple system atrophy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The case of a 38-year-old patient with rapidly progressing motor neuron disease, complicated by major dysfunction of the extrapyramidal system and of vertical gaze is described. Neuropathological examination revealed a degenerative process that severely affected the lower motor neurons, as well as the neurons of the pars compacta of the substantia nigra, the nucleus of Darkschewitsch, the nucleus interstitialis of Cajal, the colliculi superiores, and the pallidum. The long tracts were unaffected at all levels of the brain stem and spinal cord. There was no convincing evidence for the presence of a multiple system atrophy or progressive supranuclear palsy; the results rather revealed a pattern of vulnerability characteristic of a variant of motor neuron disease.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Glutamate-mediated neurotoxicity and a reduced expression of the excitatory amino acid transporter 2 (EAAT2) have been described in the pathogenesis of several acute and chronic neurological conditions. EAAT2 is the major carrier of glutamate in the mammalian brain. However, the principles of EAAT2 expression regulation are not fully understood. For the human brain, extensive alternative splicing of the EAAT2 RNA has been shown. To delineate the complex RNA regulation of EAAT2 we investigated whether the murine species is a suitable model for the study of EAAT2 splicing events. We identified five splice variants (mEAAT2/5UT1–5) encoding different 5′-untranslated sequences and two distinct N-termini of the putative EAAT2 polypeptide. In the murine CNS we found a region-specific expression pattern of the novel 5′-variants of EAAT2 as shown by in situ hybridization, dot blotting and competitive reverse transcription polymerase chain reaction. Furthermore, we performed an expression analysis of the EAAT2 splice variants in the spinal cord of a transgenic model (SOD1G93A) of amyotrophic lateral sclerosis, a motor neurone disease for which altered splicing of EAAT2 has been discussed. We found an increased expression of mEAAT2/5UT4 and a reduction of mEAAT2/5UT5 in the early course of the disease. We conclude that alternative splicing of 5′-sequences may contribute to the regional expression of the EAAT2 RNA and was altered in the pre-symptomatic stage of the SOD1G93A-mouse model for amyotrophic lateral sclerosis.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1471-4159
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Abstract: Riluzole is neuroprotective in patients with amyotrophiclateral sclerosis and may also protect dopamine (DA) neurons in Parkinson'sdisease. We examined the neuroprotective potential of riluzole on DA neuronsusing primary rat mesencephalic cultures and human dopaminergic neuroblastomaSH-SY5Y cells. Riluzole (up to 10 μM) alone affected neither thesurvival of DA neurons in primary cultures nor the growth of SH-SY5Y cellsafter up to 72 h. Riluzole (1-10 μM) dose-dependently reduced DAcell loss caused by exposure to MPP+ in both types of cultures.These protective effects were accompanied by a dose-dependent decrease ofintracellular ATP depletion caused by MPP+ (30-300 μM)in SH-SY5Y cells without affecting intracellular net NADH content, suggestinga reduction of cellular ATP consumption rather than normalization ofmitochondrial ATP production. Riluzole (1-10 μM) also attenuatedoxidative injury in both cell types induced by exposure to L-DOPA and6-hydroxydopamine, respectively. Consistent with its antioxidative effects,riluzole reduced lipid peroxidation induced by Fe3+ and L-DOPA inprimary mesencephalic cultures. Riluzole (10 μM) did not alterhigh-affinity uptake of either DA or MPP+. However, in the samecell systems, riluzole induced neuronal and glial cell death withconcentrations higher than those needed for maximal protective effects(≥100 μM). These data demonstrate that riluzole has protectiveeffects on DA neurons in vitro against neuronal injuries induced by (a)impairment of cellular energy metabolism and/or (b) oxidative stress. Theseresults provide further impetus to explore the neuroprotective potential ofriluzole in Parkinson's disease.
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  • 4
    ISSN: 1432-1459
    Keywords: Key words Familiar amyotrophic lateral sclerosis ; Copper-zinc superoxide dismutase ; D90A
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Mutations of the SOD1 gene encoding the free radical scavenging enzyme copper-zinc superoxide dismutase (CuZn-SOD) occur in patients with familial amyotrophic lateral sclerosis (ALS). Recent reports have shown homozygosity for a CuZn-SOD mutation in exon 4, the D90A (Asp90Ala) mutation. Other mutations described to date show an autosomal dominant pattern of inheritance. This is the first description of autosomal recessively inherited ALS in an out-bred population in central Europe. This study confirms the earlier described characteristic phenotype reported in D90A homozygous ALS patients in Scandinavia and supports the theory of the existence of a strong modifying factor in some cases of ALS associated with mutations in the CuZn-SOD gene.
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  • 5
    ISSN: 1432-1459
    Keywords: Key words Amyotrophic lateral sclerosis ; Cu/Zn SOD ; EAAT2 ; AMPA ; Neurolathyrism ; Riluzole
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract It is well accepted that excitotoxic mechanisms contribute to the pathogenesis of acute neuronal death in stroke, epilepsy, or brain trauma. It is less widely acknowledged that excitotoxic mechanisms play a role in the pathogenesis of chronic neurological disorders, in particular neurodegenerative diseases. However, evidence is accumulating that this mechanism is indeed part of the pathogenesis of late-onset neurodegenerative diseases. One of the clinical examples may be amyotrophic lateral sclerosis, a disease in which antiexcitotoxic strategies have neuroprotective effects in both, an established animal model and in man. In addition, there is accumulating neuropathological, pathobiochemical and pathophysiological evidence which indicates that excitotoxic mechanisms are part of the pathogenesis of the human disease and consequently part of the mechanisms explaining selective vulnerability (“pathoclisis”) in the human motor system.
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 247 (2000), S. VI13 
    ISSN: 1432-1459
    Keywords: Key words Amyotrophic lateral sclerosis ; neurodegeneration ; riluzole
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease which was thought to be untreatable. However, recent evidence in both experimental animals and men indicates that antiglutamatergic strategies are the first to have an influence on its pathogenesis and slow down the disease process. Since the effect of drugs is still small, this process cannot only be seen as a success of the present but must also be acknowledged as a basis for future developments. How will future studies be designed? They will have to take into account that the disease presumably has a long preclinical period and they will use a number of novel compounds and treatment strategies which have been shown to be effective in transgenic animal models. This also implies that we are likely to use a combination of therapies and we will try to treat patients early. The latter will be associated with the demand for a novel clinical attitude toward the diagnosis of the disease and the development of novel markers for both the preclinical period and the longitudinal course of the disease.
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