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| Identification of the prion protein allotypes which accumulate in the brain of sporadic and familial Creutzfeldt-Jakob disease patients |
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| Author(s): Silvestrini MC, Cardone F, Maras B, Pucci P, Barra D, Brunori M, Pocchiari M |
| Source: NATURE MEDICINE Volume: 3 Issue: 5 Pages: 521-525 Published: MAY 1997 |
| Times Cited: 31 References: 47 |
| Abstract: A characteristic feature of Creutzfeldt-jakob disease (CJD) is the accumulation in the brain of the amyloid protease-resistant protein PrPres. PrPres derives from a host-encoded, protease-sensitive isoform, PrPsen. Mutations of this protein are linked to familial variants of the disease, and the presence of a methionine or valine residue at the polymorphic position 129 may be critical in sporadic CJD cases. We found that in the brain of patients heterozygous for the mutation in which isoleucine is substituted for valine at codon 210 (Val210Ile), the PrPres is formed by both the wild-type and mutant PrPsen. We also found that in a sporadic CJD patient, who was heterozygous (Met/Val) at position 129, PrPres is also formed by both allotypes. These data associate transmissible spongiform encephalopathies with other amyloidosis, although the nature of the transmissible agent remains unsettled. |
| Document Type: Article |
| Language: English |
Addresses:
1. IST SUPER SANITA, VIROL LAB, I-00161 ROME, ITALY
2. CNR, CTR MOL BIOL, I-00185 ROME, ITALY
3. UNIV NAPLES, DIPARTIMENTO CHIM ORGAN & BIOL, I-80134 NAPLES, ITALY
4. UNIV ROMA LA SAPIENZA, DIPARTIMENTO SCI BIOCHIM A ROSSI FANELLI, I-00185 ROME, ITALY |
| Publisher: NATURE PUBLISHING CO, 345 PARK AVE SOUTH, NEW YORK, NY 10010-1707 |
| Subject Category: Biochemistry & Molecular Biology; Cell Biology; Medicine, Research & Experimental |
| IDS Number: WX543 |
| ISSN: 1078-8956 |
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