Search results for "prion"
showing 10 items of 53 documents
Two novel amino acid substitutions in highly conserved regions of prion protein (PrP) and a high frequency of a scrapie protective variant in native …
2019
Background Polymorphisms of the prion protein gene may influence scrapie susceptibility in small ruminants through modified protein conformation. At least 47 amino acid substitutions and 19 silent polymorphisms have been described in goat PRNP reported from several countries. The objective of this study was to investigate PRNP polymorphisms of native Ethiopian goat breeds and compare the results with other goat breeds. Results The analysis of the prion protein gene PRNP in 229 goats belonging to three of the main Ethiopian native goat breeds showed a remarkably high frequency (> 34.6%) of p.(Asn146Ser) in these breeds, a variant involved in scrapie resistance in Cyprus. In addition, two nov…
Pro-prion Binds Filamin A, Facilitating Its Interaction with Integrin β1, and Contributes to Melanomagenesis
2010
Filamin A (FLNA) is an integrator of cell mechanics and signaling. The spreading and migration observed in FLNA sufficient A7 melanoma cells but not in the parental FLNA deficient M2 cells have been attributed to FLNA. In A7 and M2 cells, the normal prion (PrP) exists as pro-PrP, retaining its glycosylphosphatidyl-inositol (GPI) anchor peptide signal sequence (GPI-PSS). The GPI-PSS of PrP has a FLNA binding motif and binds FLNA. Reducing PrP expression in A7 cells alters the spatial distribution of FLNA and organization of actin and diminishes cell spreading and migration. Integrin β1 also binds FLNA. In A7 cells, FLNA, PrP, and integrin β1 exist as two independent, yet functionally linked,…
Hymenopteran specificity of Bacillus thuringiensis strain PS86Q3
2008
Abstract The biological activity of Bacillus thuringiensis (Bt) strain PS86Q3 against five Hymenopteran species was determined by means of bioassays adapted to each species. Four species of sawfly that are important pests of conifers (Diprion pini, Gilpinia hercyniae and Pristiphora abietina) or ornamental plants (Arge rosae), as well as the non-target honeybee, Apis mellifera, were studied. Two out of the four sawfly species tested were found to be sensitive to PS86Q3 crystals or spore/crystal suspensions. A sporulated culture of this strain was moderately active on D. pini, and a complete bioassay with solubilized crystals was performed to estimate the LC50 of 4.9 mg/ml. Pristiphora abiet…
Analysis of the influence of the PrP genotype on the litter size in Polish sheep using classification trees and logistic regression
2014
The relation between prion protein (PrP) genotype class and litter size was analysed in Polish Merino, Black-headed, Ile de France and Berrichon du Cher sheep in Poland. Litter size was analysed with the classification tree technique and multiple logistic regression for effects of breed, age of ewe, PrP genotype, litter size of ewe at her birth, and at her rearing and year of ewe's birth. The resulting classification tree was 6-level deep and contained 21 leaves. Age, breed and year of birth were more important determinants of litter size than PrP genotype. Importance of PrP genotype in the tree development was 0.560 relative to age of ewe. While the logistic regression analysis showed that…
Gastric α-synuclein immunoreactive inclusions in Meissner's and Auerbach's plexuses in cases staged for Parkinson's disease-related brain pathology
2005
The progressive degenerative process associated with sporadic Parkinson's disease (sPD) is characterized by formation of alpha-synuclein-containing inclusion bodies in a few types of projection neurons in both the enteric and central nervous systems (ENS and CNS). In the brain, the process apparently begins in the brainstem (dorsal motor nucleus of the vagal nerve) and advances through susceptible regions of the basal mid-and forebrain until it reaches the cerebral cortex. Anatomically, all of the vulnerable brain regions are closely interconnected. Whether the pathological process begins in the brain or elsewhere in the nervous system, however, is still unknown. We therefore used immunocyt…
Mathematical models for the diffusion magnetic resonance signal abnormality in patients with prion diseases
2014
In clinical practice signal hyperintensity in the cortex and/or in the striatum on magnetic resonance (MR) diffusion-weighted images (DWIs) is a marker of sporadic Creutzfeldt–Jakob Disease (sCJD). MR diagnostic accuracy is greater than 90%, but the biophysical mechanisms underpinning the signal abnormality are unknown. The aim of this prospective study is to combine an advanced DWI protocol with new mathematical models of the microstructural changes occurring in prion disease patients to investigate the cause of MR signal alterations. This underpins the later development of more sensitive and specific image-based biomarkers. DWI data with a wide a range of echo times and diffusion weightin…
Quantitative and integrative proteome analysis of peripheral nerve myelin identifies novel myelin proteins and candidate neuropathy loci
2011
Peripheral nerve myelin facilitates rapid impulse conduction and normal motor and sensory functions. Many aspects of myelin biogenesis, glia–axonal interactions, and nerve homeostasis are poorly understood at the molecular level. We therefore hypothesized that only a fraction of all relevant myelin proteins has been identified so far. Combining gel-based and gel-free proteomic approaches, we identified 545 proteins in purified mouse sciatic nerve myelin, including 36 previously known myelin constituents. By mass spectrometric quantification, the predominant P0, periaxin, and myelin basic protein constitute 21, 16, and 8% of the total myelin protein, respectively, suggesting that their relat…
<p><strong>Revision of the genus <em>Prionotropis</em> Fieber, 1853 (Orthoptera: Pamphagidae: Thrinchinae)</strong><…
2015
The genus Prionotropis Fieber, 1853 is revised. It is distributed in scattered areas of the Mediterranean region from Turkey in the East to Spain in the West. Overall, seven species are listed, namely P. maculinervis (Stal, 1878) (Turkey; P. urfensis Ramme, 1933 is here considered its synonym), P. willemsorum n. sp. (Greece, Epirus; previously considered P. appula), P. appula (O.G. Costa, 1836) (South Italy), P. hystrix (Germar, 1817) (Bosnia and Herzegovina, Slovenia, Croatia, North-East Italy; P. hystrix sontiaca is here synonymized), P. rhodanica Uvarov, 1923 resurrected status (France, Crau, Rhone delta; here considered a valid species), P. azami Uvarov, 1923 n. status (France, Var regi…
First historical records of Carcharhinus brachyurus (Chondrichthyes, Carcharhiniformes) in the Mediterranean Sea
2009
The dried jaws of two specimens of Carcharhinus brachyurus were found in the collections of the Zoological Museum of the University of Palermo. Both pieces belong to the great Doderlein collection of fishes from Sicily assembled during the end of the nineteenth century (1862-1892) and are labelled as Carcarias (Prionodon) lamia and Carcharias lamia, respectively. These findings represent the first historical evidence of the presence of C. brachyurus in the Mediterranean Sea and add the southern Tyrrhenian to the species distribution within the Mediterranean. Moreover, sexual dimorphism in tooth morphology is documented for the first time in Mediterranean specimens. Some meristic and morphol…
Prions, mad cow disease, and preventive measures: a critical appraisal
2003
In 1996 the first key epidemiological study on bovine spongiform encephalitis (BSE) appeared in the renowned journal Nature [1]. In that article it was estimated that by the year 1996, some 750,000 cows with BSE had entered the food chain in Great Britain. Accordingly, millions of people in GB must have consumed contaminated meat. That same year the first report on a new form of Creutzfeldt-Jakob disease [variant (v) CJD] manifesting in young patients appeared [26]. A connection between this disease and BSE was assumed. In view of the suspicion that the use of meat and bone meal (MBM) had led to the outbreak of BSE, feeding with MBM was banned in the year 1988. The number of new BSE infecti…