Four types of scrapie in goats differentiated from each other and bovine spongiform encephalopathy by biochemical methods

J.P.M. Langeveld*, L. Pirisinu, J.G. Jacobs, M. Mazza, Isabelle Lantier, Stephanie Simon, Olivier Andrèoletti, C. Acin, E. Esposito, C. Fast, Martin H. Groschup, Wilfred Goldmann, J. Spiropoulos, Theodoros Sklaviadis, F. Lantier, Loukia V. Ekateriniadou, P. Papasavva-Stylianou, L.J.M. van Keulen, Pier Luigi Acutis, U. Agrimi & 2 others A. Bossers, Romolo Nonno

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

1 Citation (Scopus)

Abstract

Scrapie in goats has been known since 1942, the archetype of prion diseases in which only prion protein (PrP) in misfolded state (PrPSc) acts as infectious agent with fatal consequence. Emergence of bovine spongiform encephalopathy (BSE) with its zoonotic behaviour and detection in goats enhanced fears that its source was located in small ruminants. However, in goats knowledge on prion strain typing is limited. A European-wide study is presented concerning the biochemical phenotypes of the protease resistant fraction of PrPSc (PrPres) in over thirty brain isolates from transmissible spongiform encephalopathy (TSE) affected goats collected in seven countries. Three different scrapie forms were found: classical scrapie (CS), Nor98/atypical scrapie and one case of CH1641 scrapie. In addition, CS was found in two variants—CS-1 and CS-2 (mainly Italy)—which differed in proteolytic resistance of the PrPres N-terminus. Suitable PrPres markers for discriminating CH1641 from BSE (C-type) appeared to be glycoprofile pattern, presence of two triplets instead of one, and structural (in)stability of its core amino acid region. None of the samples exhibited BSE like features. BSE and these four scrapie types, of which CS-2 is new, can be recognized in goats with combinations of a set of nine biochemical parameters.
Original languageEnglish
Article number97
JournalVeterinary Research
Volume50
DOIs
Publication statusPublished - 25 Nov 2019

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Bovine Spongiform Encephalopathy
Scrapie
bovine spongiform encephalopathy
scrapie
Goats
goats
PrPSc proteins
Prion Diseases
prion diseases
methodology
prions
Prions
Zoonoses
Ruminants
small ruminants
fearfulness
Italy
Fear
Peptide Hydrolases
proteinases

Cite this

Langeveld, J. P. M., Pirisinu, L., Jacobs, J. G., Mazza, M., Lantier, I., Simon, S., ... Nonno, R. (2019). Four types of scrapie in goats differentiated from each other and bovine spongiform encephalopathy by biochemical methods. Veterinary Research, 50, [97]. https://doi.org/10.1186/s13567-019-0718-z
Langeveld, J.P.M. ; Pirisinu, L. ; Jacobs, J.G. ; Mazza, M. ; Lantier, Isabelle ; Simon, Stephanie ; Andrèoletti, Olivier ; Acin, C. ; Esposito, E. ; Fast, C. ; Groschup, Martin H. ; Goldmann, Wilfred ; Spiropoulos, J. ; Sklaviadis, Theodoros ; Lantier, F. ; Ekateriniadou, Loukia V. ; Papasavva-Stylianou, P. ; van Keulen, L.J.M. ; Acutis, Pier Luigi ; Agrimi, U. ; Bossers, A. ; Nonno, Romolo. / Four types of scrapie in goats differentiated from each other and bovine spongiform encephalopathy by biochemical methods. In: Veterinary Research. 2019 ; Vol. 50.
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abstract = "Scrapie in goats has been known since 1942, the archetype of prion diseases in which only prion protein (PrP) in misfolded state (PrPSc) acts as infectious agent with fatal consequence. Emergence of bovine spongiform encephalopathy (BSE) with its zoonotic behaviour and detection in goats enhanced fears that its source was located in small ruminants. However, in goats knowledge on prion strain typing is limited. A European-wide study is presented concerning the biochemical phenotypes of the protease resistant fraction of PrPSc (PrPres) in over thirty brain isolates from transmissible spongiform encephalopathy (TSE) affected goats collected in seven countries. Three different scrapie forms were found: classical scrapie (CS), Nor98/atypical scrapie and one case of CH1641 scrapie. In addition, CS was found in two variants—CS-1 and CS-2 (mainly Italy)—which differed in proteolytic resistance of the PrPres N-terminus. Suitable PrPres markers for discriminating CH1641 from BSE (C-type) appeared to be glycoprofile pattern, presence of two triplets instead of one, and structural (in)stability of its core amino acid region. None of the samples exhibited BSE like features. BSE and these four scrapie types, of which CS-2 is new, can be recognized in goats with combinations of a set of nine biochemical parameters.",
author = "J.P.M. Langeveld and L. Pirisinu and J.G. Jacobs and M. Mazza and Isabelle Lantier and Stephanie Simon and Olivier Andr{\`e}oletti and C. Acin and E. Esposito and C. Fast and Groschup, {Martin H.} and Wilfred Goldmann and J. Spiropoulos and Theodoros Sklaviadis and F. Lantier and Ekateriniadou, {Loukia V.} and P. Papasavva-Stylianou and {van Keulen}, L.J.M. and Acutis, {Pier Luigi} and U. Agrimi and A. Bossers and Romolo Nonno",
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Langeveld, JPM, Pirisinu, L, Jacobs, JG, Mazza, M, Lantier, I, Simon, S, Andrèoletti, O, Acin, C, Esposito, E, Fast, C, Groschup, MH, Goldmann, W, Spiropoulos, J, Sklaviadis, T, Lantier, F, Ekateriniadou, LV, Papasavva-Stylianou, P, van Keulen, LJM, Acutis, PL, Agrimi, U, Bossers, A & Nonno, R 2019, 'Four types of scrapie in goats differentiated from each other and bovine spongiform encephalopathy by biochemical methods', Veterinary Research, vol. 50, 97. https://doi.org/10.1186/s13567-019-0718-z

Four types of scrapie in goats differentiated from each other and bovine spongiform encephalopathy by biochemical methods. / Langeveld, J.P.M.; Pirisinu, L.; Jacobs, J.G.; Mazza, M.; Lantier, Isabelle; Simon, Stephanie; Andrèoletti, Olivier; Acin, C.; Esposito, E.; Fast, C.; Groschup, Martin H.; Goldmann, Wilfred; Spiropoulos, J.; Sklaviadis, Theodoros; Lantier, F.; Ekateriniadou, Loukia V.; Papasavva-Stylianou, P.; van Keulen, L.J.M.; Acutis, Pier Luigi; Agrimi, U.; Bossers, A.; Nonno, Romolo.

In: Veterinary Research, Vol. 50, 97, 25.11.2019.

Research output: Contribution to journalArticleAcademicpeer-review

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T1 - Four types of scrapie in goats differentiated from each other and bovine spongiform encephalopathy by biochemical methods

AU - Langeveld, J.P.M.

AU - Pirisinu, L.

AU - Jacobs, J.G.

AU - Mazza, M.

AU - Lantier, Isabelle

AU - Simon, Stephanie

AU - Andrèoletti, Olivier

AU - Acin, C.

AU - Esposito, E.

AU - Fast, C.

AU - Groschup, Martin H.

AU - Goldmann, Wilfred

AU - Spiropoulos, J.

AU - Sklaviadis, Theodoros

AU - Lantier, F.

AU - Ekateriniadou, Loukia V.

AU - Papasavva-Stylianou, P.

AU - van Keulen, L.J.M.

AU - Acutis, Pier Luigi

AU - Agrimi, U.

AU - Bossers, A.

AU - Nonno, Romolo

PY - 2019/11/25

Y1 - 2019/11/25

N2 - Scrapie in goats has been known since 1942, the archetype of prion diseases in which only prion protein (PrP) in misfolded state (PrPSc) acts as infectious agent with fatal consequence. Emergence of bovine spongiform encephalopathy (BSE) with its zoonotic behaviour and detection in goats enhanced fears that its source was located in small ruminants. However, in goats knowledge on prion strain typing is limited. A European-wide study is presented concerning the biochemical phenotypes of the protease resistant fraction of PrPSc (PrPres) in over thirty brain isolates from transmissible spongiform encephalopathy (TSE) affected goats collected in seven countries. Three different scrapie forms were found: classical scrapie (CS), Nor98/atypical scrapie and one case of CH1641 scrapie. In addition, CS was found in two variants—CS-1 and CS-2 (mainly Italy)—which differed in proteolytic resistance of the PrPres N-terminus. Suitable PrPres markers for discriminating CH1641 from BSE (C-type) appeared to be glycoprofile pattern, presence of two triplets instead of one, and structural (in)stability of its core amino acid region. None of the samples exhibited BSE like features. BSE and these four scrapie types, of which CS-2 is new, can be recognized in goats with combinations of a set of nine biochemical parameters.

AB - Scrapie in goats has been known since 1942, the archetype of prion diseases in which only prion protein (PrP) in misfolded state (PrPSc) acts as infectious agent with fatal consequence. Emergence of bovine spongiform encephalopathy (BSE) with its zoonotic behaviour and detection in goats enhanced fears that its source was located in small ruminants. However, in goats knowledge on prion strain typing is limited. A European-wide study is presented concerning the biochemical phenotypes of the protease resistant fraction of PrPSc (PrPres) in over thirty brain isolates from transmissible spongiform encephalopathy (TSE) affected goats collected in seven countries. Three different scrapie forms were found: classical scrapie (CS), Nor98/atypical scrapie and one case of CH1641 scrapie. In addition, CS was found in two variants—CS-1 and CS-2 (mainly Italy)—which differed in proteolytic resistance of the PrPres N-terminus. Suitable PrPres markers for discriminating CH1641 from BSE (C-type) appeared to be glycoprofile pattern, presence of two triplets instead of one, and structural (in)stability of its core amino acid region. None of the samples exhibited BSE like features. BSE and these four scrapie types, of which CS-2 is new, can be recognized in goats with combinations of a set of nine biochemical parameters.

U2 - 10.1186/s13567-019-0718-z

DO - 10.1186/s13567-019-0718-z

M3 - Article

VL - 50

JO - Veterinary Research

JF - Veterinary Research

SN - 0928-4249

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