Prion diseases, also known as Transmissible Spongiform Encephalopathies (TSE) are considered as fatal neurodegenerative and infectious disorders that are found in ruminants, cats, mink and humans. ). Initial researches and studies suggested that there was a virus that caused infection that caused prion diseases. PRNP is the only gene that has been identified in mammals that has been linked to prion diseases including like GSS, CWD, CJD, BSE and Scrapie. It has been signified that there is a number of octapeptide repeats in the mammalian PrP. It has been observed that most of the families use the stop codon TGA, while others use the TAA or TAG. Total 266 haplotypes have been identified that were gathered from a total of 937 sequences of various families. These were aligned by the BIOEDIT spanning of 996 BP. The result that was gathered from the DNASP analysis showed that the region that was selected of 266 haplotypes based over different species had 383 sites. The functional ability of the PrPC that is a normal cellular prion protein as a potential cell surface receptor is modulated by the removal of the N-terminal region of the protein and the proteolytic cleavage. The association studies demonstrate that there was no evidence of the pleiotropic e?ect on the Salmonella resistance, carcass and wool traits of the PRNP gene. The results identified that a genetic determinant in PRNP may have the ability to influence the susceptibility of cattle to BSE. It has been suggested from the study that the identification of the cattle involved with genetic risk factors is very important for their removal from the population of the livestock.
ABSTRACT2
3
INTRODUCTION4
Prion Diseases: A Historical Perspective4
1.LITERATURE REVIEW5
1.1.The Transmissible Agent Of Prion Diseases5
1.2.Prion Disease And The PRNP Gene6
1.3.Sequence Length Variation within and among Species8
1.4.Stop Codon Variation among Different Families9
1.5.Phylogenetic Relationship of Families and Species10
1.6.Genetic Diversity within and among Species and Families12
2.MATERIALS AND METHODS15
2.2. Introduction15
2.3. Research methods15
2.4. Method of Inquiry16
2.5. Research Method and Appropriateness16
2.6. Research Design17
2.7. Study Animals19
2.8. DNA isolation and PRNP amplification19
2.9. Sequencing20
2.10. Validation Techniques20
2.11. Computer Software And Analysis21
3.RESULTS21
3.1.Association analyses21
4.DISCUSSION25
5.LITERATURE BASED REPORTS26
5.1.Composition of atypical BSE group30
6. CONCLUSION31
REFERENCES32
Introduction
Prion Diseases: A Historical Perspective
Prion diseases are also known as TSEs (transmissible Spongiform Encephalopathies). These diseases are referred to as a group of diseases that are neurodegenerative by which humans, wild animals and domestic animals are affected (Collinge 2001). It has been observed through history that the most common names of prion diseases are found to be sub acute spongiform encephalopathies, transmissible dementias or slow viruses (Collinge 2001). A TSE that is naturally found in goats and sheep all over the world is known as Scrapie, and is considered to be a prototypical TSE. It is also observed that this disease is also sigjnificantly recognized in Europe for more than 200 years (Collinge 2001; McGowan 1922). It is also significant that additional disease of TSEs found in animals is also found to be in elk and deer including the disease of chronic wasting ...