Creutzfeldt‐Jakob disease after pituitary‐derived human growth hormone therapy

Masson, Catherine; Delalande, I; Deslys, Jean Philippe; Hénin, Dominique; Fallet-Bianco, Catherine; Dormont, Dominique; Leys, D.

doi:10.1212/wnl.44.1.179

Cited by 16 publications

(5 citation statements)

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“…We have also stopped preparing human dura mater grafts that might be infected with prions (Thandani et al 1988;Masson et al 1994;Yamada et al 1994).…”

Section: Introductionmentioning

confidence: 99%

Irradiation as a Safety Procedure in Tissue Banking

Dziedzic‐Goclawska

Kamiński

Uhrynowska-Tyszkiewicz

et al. 2005

Cell Tissue Banking

168

118

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The Central Tissue Bank in Warsaw was established in 1963 and since then ionising radiation has been routinely applied to sterilise tissue grafts. Connective tissue grafts such as bone, cartilage, tendons, sclera, pericardium, skin, acellular dermis and amnion irradiated with a dose of 35 kGy in a (60)Co source and/or with an electron beam 10 MeV accelerator are prepared in our Tissue Bank and two other multi-tissue banks operating in Poland. Over 250,000 radiation-sterilised tissue grafts have been prepared and used in hospitals throughout Poland and no infectious disease transmission or other adverse post-transplantation reactions have been reported up to today. It should be kept in mind however, that high doses of ionising radiation can evoke numerous chemical and physical changes that may affect the biological quality of tissue allografts. Therefore, interdisciplinary research has been undertaken at the Central Tissue Bank in Warsaw to establish the origin and stability of free radicals and other paramagnetic entities induced by irradiation in bone. The effects of various preservation procedures (e.g. lyophilisation, deep-freezing) and irradiation conditions (doses, temperature of irradiation) on the osteoinductive potential and mechanical properties of bone and on the degradation of collagen, a major constituent of all connective tissue grafts, have been also studied. The results of these studies indicate that radiation-induced changes can be diminished by modification of tissue preservation methods and that, to some extent, it is possible to reduce undesired radiation-induced damage to the tissue grafts.

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“…We have also stopped preparing human dura mater grafts that might be infected with prions (Thandani et al 1988;Masson et al 1994;Yamada et al 1994).…”

Section: Introductionmentioning

confidence: 99%

Irradiation as a Safety Procedure in Tissue Banking

Dziedzic‐Goclawska

Kamiński

Uhrynowska-Tyszkiewicz

et al. 2005

Cell Tissue Banking

168

118

View full text Add to dashboard Cite

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“…Also the incidence of iatrogenic transmission of Creutzfeld–Jakob disease by growth hormone treatment has been associated with polymorphisms of the PrP gene. Early in 1993, a homozygous genotype with a valine at position 129 of the PrP gene was described as playing a role in the disease incidence in French patients treated with extractive growth hormone (Labauge et al ., 1993; Masson et al ., 1994). The molecular mechanism by which these different forms of the PrP protein influence incubation times and phenotypic expression remains unknown, although thermodynamic and kinetic models have been proposed to explain the role of these single mutations (Cohen, 1999).…”

Section: Introductionmentioning

confidence: 99%

The yeast prion [URE3] can be greatly induced by a functional mutated URE2 allele

2000

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The non-Mendelian element [URE3] of yeast is considered to be a prion form of the Ure2 protein. The [URE3] phenotype occurs at a frequency of 10 ±5 in haploid yeast strains, is reversible, and its frequency is increased by overexpressing the URE2 gene. We created a new mutant of the Ure2 protein, called H2p, which results in a 1000-fold increase in the rate of [URE3] occurrence. To date, only the overexpression of various C-terminal truncated mutants of Ure2p gives rise to a comparable level. The h2 allele is, thus, the ®rst characterized URE2 allele that induces prion formation when expressed at a low level. By shuf¯ing mutated and wild-type domains of URE2, we also created the ®rst mutant Ure2 protein that is functional and induces prion formation. We demonstrate that the domains of URE2 function synergistically in cis to induce [URE3] formation, which highlights the importance of intramolecular interactions in Ure2p folding. Additionally, we show using a green¯uores-cent protein (GFP) fusion protein that the h2 allele exhibits numerous ®liform structures that are not generated by the wild-type protein.

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“…14 We have reviewed five cases with detailed neuropsychology data. All presented with a cerebellar syndrome and only one had noticed mild memory problems.…”

Section: Discussionmentioning

confidence: 99%

“…[11][12][13][14] The first published case, of a 22 year old British woman, is, however, the only report to provide formal neuropsychology data, aquired seven months from the onset of the cerebellar syndrome. 5 15 This showed evidence of intellectual decline, with a verbal intelligence quotient (VIQ) of 80.…”

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confidence: 99%