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1

Apoptosis in relation to neuronal loss in experimental Creutzfeldt-Jakob disease in mice

100%

Jesionek-Kupnicka D., Kordek R., Buczynski J., Liberski P. P.

Acta Neurobiologiae Experimentalis

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2001

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vol. 61

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issue 1

13-19

EN

Apoptosis constitutes a genetically determined process to eliminate superfluous or damaged cells in tissues. Deficiencies in apoptosis regulation are involved in different pathologies including prion diseases. Some experimental studies show that neuronal loss - one of the hallmarks of prion diseases may be accomplished by apoptosis. We evaluated twenty five mice infected experimentally with the Fujisaki strains of CJD and sacrified sequentially in one week intervals. Apoptotic cells in various brain regions were detected by in situ end labelling (TUNEL) and electron microscopy in comparison with neuronal cell loss. The number of labelled cells per brain was very low - from a few labelled cells 6 weeks after inoculation to a maximum of 14 in the terminal stage. The number of neurones counted in 8 selected areas were considerably lower in terminally sick animals (20 and 21 week of incubation period) than in control mice. The mean value of loss of neuronal cells was 32%. The greatest loss (55%) of neurones was noted in the septal nuclei of the paraterminal body and the least lost (16%) in the hypothalamus. Compared to the extensive neuronal loss (30-50%), the number of apoptotic cells detected by in situ end labelling seems to be very low, and the process of neuronal death become more intensive during the progression of the disease.

2

Codon 129 polymorphism of the PRNP gene in normal Polish population and in Creutzfeldt-Jakob disease, and? the search for new mutations in PRNP gene?

100%

Bratosiewicz J., Liberski P. P., Kulczycki J., Kordek R.

Acta Neurobiologiae Experimentalis

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2001

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vol. 61

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issue 3

151-156

EN

Polymorphism at codon 129 of the prion protein gene (PRNP) is implicated both in susceptibility and phenotype of human prion diseases. We characterized the valine and methionine allele frequency at codon 129 in 109 individuals representing the normal Polish population and in 15 Polish CJD cases. The distribution of the genotype was 45% Met/Met, 39% Met/Val, and 16% Val/Val in the control group whereas, of the CJD cases, 73.3% were homozygous for methionine, 13.3% homozygous for valine and 13.3% were heterozygous. The novel missense mutation (ATGACG) at codon 232 was identified in one of the samples with a GSS phenotype.

3

Molecular analysis of prion protein (PrP) and glial fibrillary acidic protein (GFAP) transcripts in experimental Creutzfeldt Jakob disease in mice

88%

Kordek R., Liberski P. P., Yanagihra R., Isaacson S., Gajdusek D. C.

Acta Neurobiologiae Experimentalis

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1997

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vol. 57

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issue 2

85-90

EN

Prion protein (PrPsc) which accumulates in the brains affected with subacute spongiform encephalopathies (SSE) is altered isoform of normal, cellular isoform (PrPc), and PrP deposition is accompanied with spongiosis and astrogliosis. To find the amounts of PrP and GFAP transcripts during progression of experimental Creutzfeldt Jakob disease we performed comparative RT PCR on the terminally sick mice brains, 22 weeks following inoculation with Fujisaki strain of CJD agent, and on control brains. The intensity of bands for PrP mRNA and control beta actin were similar for infected and uninfected brains, while amounts of transcripts for GFAP increased as for cytokines released by glial cells TNF-alpha and IL-1 alpha. This study supports thesis that PrPc to PrPsc conversion is post translational process not related to PrP overproduction. Increased amounts of GFAP mRNA during the course of the disease correlated with astrocytosis estimated by immunohistochemistry with anti GFAP antibody.

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3 Acta Neurobiologiae Experimentalis

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3 Kordek R.

3 Liberski P. P.

1 Bratosiewicz J.

1 Buczynski J.

1 Gajdusek D. C.

1 Isaacson S.

1 Jesionek-Kupnicka D.

1 Kulczycki J.

1 Yanagihra R.

Apoptosis in relation to neuronal loss in experimental Creutzfeldt-Jakob disease in mice

Codon 129 polymorphism of the PRNP gene in normal Polish population and in Creutzfeldt-Jakob disease, and? the search for new mutations in PRNP gene?

Molecular analysis of prion protein (PrP) and glial fibrillary acidic protein (GFAP) transcripts in experimental Creutzfeldt Jakob disease in mice