Atypical BSE in Germany—Proof of transmissibility and biochemical characterization

Buschmann, A.; Gretzschel, Anja; Biacabe, Anne-Gaëlle; Schiebel, Katrin; Corona, Cristiano; Hoffmann, Christine; Eiden, Martin; Baron, TH; Casalone, Cristina; Groschup, Martin H.

doi:10.1016/j.vetmic.2006.06.016

Cited by 161 publications

(242 citation statements)

References 27 publications

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“…A similar low-molecular-mass PrP res profile has been observed in L-type BSE or BASE ( 11 , 13 , 16 ), and in the current study, these similar PrP res molecular properties between bovine TME and L-type BSE were demonstrated in TgOvPrP4 mice. These 2 distinct bovine TSE sources were both readily transmitted into TgOvPrP4 mice (illustrating the usefulness of transgenic mouse models for prion agent strain typing when transmission to a common wild-type rodent is not possible) and had several common features including survival periods, PrP res molecular features, and the distribution of vacuolar pathologic changes.…”

Section: Discussionsupporting

confidence: 88%

“…The lower molecular mass of the unglycosylated PrP res polypeptide in L-type BSE was maintained in TgOvPrP4 mice as well as in typical BSE. In cattle, the size of the unglycosylated PrP res polypeptide in L-type BSE is within the range of that found in typical BSE ( 16 ). Notably, our findings did not produce evidence for modification of the phenotypic features of these cattle TSEs on passage into TgOvPrP4 mice, which was recently described after transmission of L-type BSE into wild-type ( 18 ) or tg338 ovine transgenic ( 24 ) mice.…”

Section: Discussionsupporting

confidence: 57%

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Phenotypic Similarity of Transmissible Mink Encephalopathy in Cattle and L-type Bovine Spongiform Encephalopathy in a Mouse Model

Baron

Bencsik

Biacabe

et al. 2007

Emerg. Infect. Dis.

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Section: Discussionsupporting

confidence: 88%

Section: Discussionsupporting

confidence: 57%

Phenotypic Similarity of Transmissible Mink Encephalopathy in Cattle and L-type Bovine Spongiform Encephalopathy in a Mouse Model

Baron

Bencsik

Biacabe

et al. 2007

Emerg. Infect. Dis.

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“…L-type PrP res molecules differed from zoonotic BSE by a They are associated with zoonotic ('classical') BSE cases and uncommon ('atypical') BSE cases (Ltype or H-type). These agents maintain distinct properties on serial passage in mice transgenic for bovine PrP (Bov) supporting the existence of at least three distinct prion strains in cattle [21,42,45]. Their potential of interspecies transmission has been assessed with transgenic mice expressing sheep VRQ (Ov VRQ ) or ARQ allele (Ov ARQ ) or human (MM allele, Hu Met ) PrP.…”

Section: New Strains Can Emerge From Classical and Atypical Bse Agentsmentioning

confidence: 99%

Prion agent diversity and species barrier

2008

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-Mammalian prions are the infectious agents responsible for transmissible spongiform encephalopathies (TSE), a group of fatal, neurodegenerative diseases, affecting both domestic animals and humans. The most widely accepted view to date is that these agents lack a nucleic acid genome and consist primarily of PrP Sc , a misfolded, aggregated form of the host-encoded cellular prion protein (PrP C ) that propagates by autocatalytic conversion and accumulates mainly in the brain. The BSE epizooty, allied with the emergence of its human counterpart, variant CJD, has focused much attention on two characteristics that prions share with conventional infectious agents. First, the existence of multiple prion strains that impose, after inoculation in the same host, specific and stable phenotypic traits such as incubation period, molecular pattern of PrP Sc and neuropathology. Prion strains are thought to be enciphered within distinct PrP Sc conformers. Second, a transmission barrier exists that restricts the propagation of prions between different species. Here we discuss the possible situations resulting from the confrontation between species barrier and prion strain diversity, the molecular mechanisms involved and the potential of interspecies transmission of animal prions, including recently discovered forms of TSE in ruminants.prion / strain / misfolding / species barrier / PrP Table of Mammalian prion diseases or transmissible spongiform encephalopathies (TSE) form a group of related, invariably fatal neurodegenerative disorders of both animals and humans. The brain pathology consists of spongiosis, astrocytosis, neuronal loss and neural tissue from affected individuals contains an infectious agent, the prion, setting these diseases apart from other neurodegenerative diseases. Prion replication is thought to involve in essence the self-perpetuating conversion of the host-encoded cellular prion protein (PrP C ) into a misfolded form (PrP Sc ) that tends to aggregate and may be neurotoxic (for reviews [1,157]). Prion replication may also occur in lymphoid tissues but is there poorly if not pathogenic (for review [126], [133]). PrP C is a protein with two variably occupied glycosylation sites. It is attached at the outer of the plasma membrane by a glycosylphosphatidylinositol anchor. Its secondary structure is rich in alpha-helix and the protein is likely to be in a monomeric state in mild detergents. While its precise physiological function has not been assigned, PrP C is essential for prion replication and neurotoxicity to occur [57,129]. Upon infection, PrP C is refolded -without apparent post-translational modification -into beta-sheet -rich PrP Sc , initially in the presence of exogenous PrP Sc and then by an autocatalytic process. It leads to the formation of aggregates, sometimes of amyloid type, that can be differentiated from PrP C because of their partial resistance to protease digestion and of their insolubility into non-denaturing detergents [153]. Proteinase K, the most commonly used protease, di...

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“…More precisely, transmission of H-type in wild-type mice (C57Bl6) showed that the molecular features were maintained in mice [7], and transmission of H-type in transgenic mice expressing bovine or ovine PrP showed features clearly distinct from that of classical BSE as well as sheep scrapie isolates passaged in these models so far [8]. Transmission of L-type in other bovine PrP transgenic mice led to an incubation period significantly shorter than that found with classical BSE and showing distinct biochemical characteristics [14]. Experimental transmission of the two types to cattle has been initiated recently 14 and results will be of the utmost interest.…”

Section: Atypical Bses and Their Epidemiological Meaningmentioning

confidence: 95%

“…Transmission of L-type in other bovine PrP transgenic mice led to an incubation period significantly shorter than that found with classical BSE and showing distinct biochemical characteristics [14]. Experimental transmission of the two types to cattle has been initiated recently 14 and results will be of the utmost interest.…”

Section: Atypical Bses and Their Epidemiological Meaningmentioning

confidence: 99%

Review on the epidemiology and dynamics of BSE epidemics

Ducrot¹,

et al. 2008

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-The paper describes how the comprehensive surveillance of bovine spongiform encephalopathy (BSE) and studies carried out on these data has enhanced our knowledge of the epidemiology of BSE. Around 7 000 BSE cases were detected through the screening of about 50 million cattle with rapid tests in Europe. It confirmed that the clinical surveillance had a poor capacity to detect cases, and also showed the discrepancy of this passive surveillance efficiency between regions and production types (dairy/beef). Other risk factors for BSE were being in a dairy herd (three times more than beef), having a young age at first calving (for dairy cattle), being autumn-born (dairy and beef), and being in a herd with a very high milk yield. These findings focus the risk on the feeding regimen of calves/heifers. Several epidemiological studies across countries suggest that the feedborne source related to meat and bone meal (MBM) is the only substantiated route of infection -even after the feed ban -, while it is not possible to exclude maternal transmission or milk replacers as a source of some infections. In most European countries, the average age of the cases is increasing over time and the prevalence decreasing, which reflects the effectiveness of control measures. Consistent results on the trend of the epidemic were obtained using back-calculation modelling, the R 0 approach and Age-Period-Cohort models. Furthermore, active surveillance also resulted in the finding of atypical cases. These are distinct from previously found BSE and classified in two different forms based on biochemical characteristics; their prevalence is very low (36 cases up to 1st September 2007), affected animals were old and some of them displayed clinical signs. The origin and possibility of natural transmission is unknown.

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Atypical BSE in Germany—Proof of transmissibility and biochemical characterization

Cited by 161 publications

References 27 publications

Phenotypic Similarity of Transmissible Mink Encephalopathy in Cattle and L-type Bovine Spongiform Encephalopathy in a Mouse Model

Phenotypic Similarity of Transmissible Mink Encephalopathy in Cattle and L-type Bovine Spongiform Encephalopathy in a Mouse Model

Prion agent diversity and species barrier

Review on the epidemiology and dynamics of BSE epidemics

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