Selective neuronal vulnerability is involved in cerebellar lesions of Guinea pigs infected with bovine spongiform encephalopathy (BSE) prions: Immunohistochemical and electron microscopic investigations

2019 ◽  
Vol 40 (2) ◽  
pp. 167-179
Author(s):  
Shoichi Sakaguchi ◽  
Sayo Shintani ◽  
Kyohei Kamio ◽  
Akio Sekiya ◽  
Satomi Kato ◽  
...  
2016 ◽  
Vol 90 (21) ◽  
pp. 9558-9569 ◽  
Author(s):  
Joel C. Watts ◽  
Kurt Giles ◽  
Daniel J. Saltzberg ◽  
Brittany N. Dugger ◽  
Smita Patel ◽  
...  

ABSTRACTThe biochemical and neuropathological properties of bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) prions are faithfully maintained upon transmission to guinea pigs. However, primary and secondary transmissions of BSE and vCJD in guinea pigs result in long incubation periods of ∼450 and ∼350 days, respectively. To determine if the incubation periods of BSE and vCJD prions could be shortened, we generated transgenic (Tg) mice expressing guinea pig prion protein (GPPrP). Inoculation of Tg(GPPrP) mice with BSE and vCJD prions resulted in mean incubation periods of 210 and 199 days, respectively, which shortened to 137 and 122 days upon serial transmission. In contrast, three different isolates of sporadic CJD prions failed to transmit disease to Tg(GPPrP) mice. Many of the strain-specified biochemical and neuropathological properties of BSE and vCJD prions, including the presence of type 2 protease-resistant PrPSc, were preserved upon propagation in Tg(GPPrP) mice. Structural modeling revealed that two residues near the N-terminal region of α-helix 1 in GPPrP might mediate its susceptibility to BSE and vCJD prions. Our results demonstrate that expression of GPPrP in Tg mice supports the rapid propagation of BSE and vCJD prions and suggest that Tg(GPPrP) mice may serve as a useful paradigm for bioassaying these prion isolates.IMPORTANCEVariant Creutzfeldt-Jakob disease (vCJD) and bovine spongiform encephalopathy (BSE) prions are two of the prion strains most relevant to human health. However, propagating these strains in mice expressing human or bovine prion protein has been difficult because of prolonged incubation periods or inefficient transmission. Here, we show that transgenic mice expressing guinea pig prion protein are fully susceptible to vCJD and BSE prions but not to sporadic CJD prions. Our results suggest that the guinea pig prion protein is a better, more rapid substrate than either bovine or human prion protein for propagating BSE and vCJD prions.


Author(s):  
G. Lehmpfuhl

Introduction In electron microscopic investigations of crystalline specimens the direct observation of the electron diffraction pattern gives additional information about the specimen. The quality of this information depends on the quality of the crystals or the crystal area contributing to the diffraction pattern. By selected area diffraction in a conventional electron microscope, specimen areas as small as 1 µ in diameter can be investigated. It is well known that crystal areas of that size which must be thin enough (in the order of 1000 Å) for electron microscopic investigations are normally somewhat distorted by bending, or they are not homogeneous. Furthermore, the crystal surface is not well defined over such a large area. These are facts which cause reduction of information in the diffraction pattern. The intensity of a diffraction spot, for example, depends on the crystal thickness. If the thickness is not uniform over the investigated area, one observes an averaged intensity, so that the intensity distribution in the diffraction pattern cannot be used for an analysis unless additional information is available.


Author(s):  
Dr. G. Kaemof

A mixture of polycarbonate (PC) and styrene-acrylonitrile-copolymer (SAN) represents a very good example for the efficiency of electron microscopic investigations concerning the determination of optimum production procedures for high grade product properties.The following parameters have been varied:components of charge (PC : SAN 50 : 50, 60 : 40, 70 : 30), kind of compounding machine (single screw extruder, twin screw extruder, discontinuous kneader), mass-temperature (lowest and highest possible temperature).The transmission electron microscopic investigations (TEM) were carried out on ultra thin sections, the PC-phase of which was selectively etched by triethylamine.The phase transition (matrix to disperse phase) does not occur - as might be expected - at a PC to SAN ratio of 50 : 50, but at a ratio of 65 : 35. Our results show that the matrix is preferably formed by the components with the lower melting viscosity (in this special case SAN), even at concentrations of less than 50 %.


Author(s):  
J. Chakraborty ◽  
A. P. Sinha Hikim ◽  
J. S. Jhunjhunwala

Although the presence of annulate lamellae was noted in many cell types, including the rat spermatogenic cells, this structure was never reported in the Sertoli cells of any rodent species. The present report is based on a part of our project on the effect of torsion of the spermatic cord to the contralateral testis. This paper describes for the first time, the fine structural details of the annulate lamellae in the Sertoli cells of damaged testis from guinea pigs.One side of the spermatic cord of each of six Hartly strain adult guinea pigs was surgically twisted (540°) under pentobarbital anesthesia (1). Four months after induction of torsion, animals were sacrificed, testes were excised and processed for the light and electron microscopic investigations. In the damaged testis, the majority of seminiferous tubule contained a layer of Sertoli cells with occasional spermatogonia (Fig. 1). Nuclei of these Sertoli cells were highly pleomorphic and contained small chromatinic clumps adjacent to the inner aspect of the nuclear envelope (Fig. 2).


2014 ◽  
Author(s):  
Noboru Manabe ◽  
Ichiro Onoyama ◽  
Junyou Li ◽  
Yutaka Sendai ◽  
Yoshito Aoyagi

1997 ◽  
Vol 141 (14) ◽  
pp. 352-357 ◽  
Author(s):  
A. R. Austin ◽  
L. Pawson ◽  
S. Meek ◽  
S. Webster

Prion ◽  
2021 ◽  
Vol 15 (1) ◽  
pp. 1-11
Author(s):  
Sandor Dudas ◽  
Renee Anderson ◽  
Antanas Staskevicus ◽  
Gordon Mitchell ◽  
James C. Cross ◽  
...  

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