Prions types

Insect cells High expression levels Free of virus or prion-type agents Can produce accurate glycosylation Difficult to scale up High mannose chains can be immunogenic... 

Keywords Animal Atypical Atypical/Nor98 scrapie BSE-H BSE-L Human Prion disease Prion strain Prion type... 

Cali I, Castellani R, Alshekhlee A et al (2009) Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt-Jakob disease its effect on the phenotype and prion-type characteristics. Brain 132 2643-2658... 

Vimses are one of the smallest biological entities (except viroids and prions) that carry all the iaformation necessary for thek own reproduction. They are unique, differing from procaryotes and eucaryotes ia that they carry only one type of nucleic acid as genetic material, which can be transported by the vims from one cell to another. Vimses are composed of a shell of proteki enclosing a core of nucleic acid, either ribonucleic acid (RNA) or deoxyribonucleic acid (DNA), that codes for vkal reproduction. The outer shell serves as a protective coat to keep the nucleic acid kitact and safe from enzymatic destmction. In addition to thek proteki coat, some vimses contain an outer covering known as an outer envelope. This outer envelope consists of a Hpid or polysaccharide material. 

Y Levy, OM Becker. Wild-type and mutant prion proteins Insights from energy landscape analysis. In E Katzir, B Solomon, A Taraboulos, eds. Conformational Diseases. In press. 

Distinct PrPSc types are seen in human prion disease 799... 

Molecular strain typing facilitates investigation of prion disease etiology 800... 

Both PrP sequence and prion strain type influence prion transmission barriers 800... 

Human prion diseases encompass the three etiological types of prion disease inherited, sporadic and acquired forms. 

PSI] is the prion of Sup35p, a translation termination factor (Ter-Avanesyan et al., 1994 Wickner, 1994). Conversion of wild-type yeast cells to the infected state results in reduction of the termination activity and, consequendy, to a nonsense suppression phenotype. This property can be used to detect [PSI] by genetic selection (Fig. 2). Sup35p is an essential gene whose knockout leads to cell death. Therefore, it appears that the [PSI] condition corresponds to only partial inactivation of Sup35p and enough of the normal protein is left to avert cell death. 

In infected cells, fungal prion proteins are aggregated. This property has been demonstrated in experiments in which the prion protein was fused with green fluorescent protein (GFP). In wild-type cells, fluorescence was homogeneously distributed throughout the cytoplasm while in cells exhibiting the prion phenotype, it was concentrated in discrete spots and/or variably shaped aggregates (Fig. 4 Coustou-Linares et al, 2001 ... 

The cause of AD is unknown although many hypotheses abound. The gene for one of the excessive amyloid proteins(b-type) has been associated with chromosome 21 at a point not far from a locus linked to some cases of familial Alzheimer s. Victims of Down s syndrome have an extra copy of chromosome 21 and usually fall prey to AD in their 4O s should they live to that age. However no all AD victims have a 21 mutation. Other causative theories involve accumulations of aluminum in the brain or the presence of a slow virus or an infectious protein substance called a prion. 

Parkinson disease, some cases of Alzheimer disease, and some types of prion disease are accompanied by the presence of Lewy bodies within the cytoplasm of neurons and also in nearby glia. These deposits consist largely of a dense core of fibrils of a-synuclein, a small 140-residue protein abundant in various parts... 

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