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It has been suggested that oxidative stress plays an important role in mediating excitotoxic neuronal death. We have therefore investigated the protective effects of antioxidants against excitotoxic injury in the rat on striatal neurons both in vitro and in vivo. In the first part of the study, we determined whether two different types of antioxidants, the(More)
Areas of striatal grafts which contain neurons that are characteristic of the striatum are called P-zones. We have investigated whether the paucity of P-zones in human xenografts of lateral ganglionic eminence (LGE) tissue in a rat model of Huntington's disease is due (i) to an absence of the appropriate target cells of LGE neurons or (ii) to the(More)
Basic parameters which are crucial for the survival of human embryonic striatal grafts need to be investigated before initiating clinical trials in Huntington's disease. In order to define the dissection of human striatal-donor tissue which gives rise to the largest amount of striatal neurons after intrastriatal transplantation, we studied the lateral and(More)
Reactive oxygen species are believed to play a crucial role in situations where dopamine neurons die, such as in Parkinson's disease or during intracerebral transplantation of embryonic mesencephalic tissue. The present study was designed to address the question whether, and to what extent, the glutathione redox system is important for the viability of rat(More)
A case of Creutzfeldt-Jakob disease (CJD) with a rare mutation of the prion protein (PrP) gene (PRNP) at codon 208 (R208H) is described. By comparison with two preceding reports, the case described here displayed two distinct biochemical and neuropathological features. Western blot analysis of brain homogenates showed, in addition to the commonly observed(More)
Sirs, Prion diseases are transmissible diseases that include sporadic, acquired and familial disorders. More than 30 point mutations and insert mutations of the prion protein gene (PRNP) are thought to cause familial prion diseases, i.e. familial Creutzfeldt-Jakob disease (fCJD), GerstmannSträussler-Scheinker syndrome (GSS) and fatal familial insomnia(More)
A heterozygous T183A mutation in the prion protein (PrP) gene, PRNP, was identified in a patient with histopathologically confirmed spongiform encephalopathy. Clinically, this form of prion disease was characterized by early-onset dementia as the predominant sign, along with global cerebral atrophy and hypometabolism. The age at onset was 40 years and the(More)
Clinical and pathological changes in familial Creutzfeldt-Jakob disease (CJD) cases may be similar or indistinguishable from sporadic CJD. Therefore determination of novel mutations in PRNP remains of major importance. We identified two different rare mutations in codon 188 of the prion protein gene (PRNP) in four patients suffering from a disease(More)
Intrastriatal grafting of embryonic dopamine-containing neurons is a promising approach for treating clinical and experimental Parkinson's disease. However, neuropathological analyses of grafted patients and transplanted rats have demonstrated that the survival of grafted dopamine neurons is relatively poor. In the present study, we pursued a strategy of(More)
Sirs, Inherited prion diseases segregate with mutations in the coding region of the human prion protein gene (PRNP). Whilst particular point mutations in the central and C-terminal moiety of the prion protein (PrP) cause the majority of familial Creutzfeldt-Jakob disease (CJD), Gerstmann-Str ussler-Scheinker syndrome, and fatal familial insomnia, base pair(More)