A Review on Protein Misfolding Diseases and therapeutics for preventing Diseases

Background: Protein misfolding sicknesses are the gathering of irresistible lethal neuro and non-neurodegenerative infections and in ebb and flow researchers and specialists accepted that unusual folding of protein is the essential or main key of such illnesses are Alzheimer's infections, Parkinson's diseases, Huntington's sickness, Creutzfeldt-Jakob infection, cystic fibrosis, Gaucher's infection and numerous other degenerative and neurodegenerative problems. The motive of this review article is to gave a detailed of the existing structural information for prion and prion protein and also we will trying to find out their preventing root causes with respect to structural information of prions within the context of what is known about the protein misfolding diseases. Objective: This article presents a brief overview of research on the use of these therapeutics for the treatment or improvement in prion diseases or protein misfolding. Material and Methods: This article begins with the brief introduction about protein misfolding diseases or infections and the therapeutic materials which are used in researches or explain this article (pentosan polysulfate, Quinacrine, Doxycycline, Chaperone based therapy, Resveratrol and curcumin) etc. Results and Conclusions: In this present context of protein misfolding/prion diseases diagonsis.Therapeutic approaches predicts that person infected with prion diseases prolongs the survival time of the patient and improvement in the conditions of the prion diseased infected patient which provides good result for future medicine development. Keywords: Amyloid, Beta-sheet, neurodegenerative, prion, protein misfolding, therapeutics etc

Familial systemic amyloidosis associated with bilateral sensorineural hearing loss and bilateral facial palsies

The Finnish type of familial amyloid polyneuropathy due to variant gelsolin is a rare form of familial amyloidosis. The subtype was first described in 1969 and is characterized by progressive cranial neuropathies, corneal lattice dystrophy and distal sensorimotor dysfunction. It is extremely uncommon, with only two families known to be affected in the UK. We discuss the case of a 70-year-old woman who presented with bilateral facial nerve palsies, bilateral sensorineural hearing loss and Finnish type familial hereditary amyloidosis. A literature search of the Medline database (1966–2005) was performed, using the keywords ‘amyloid’, ‘hearing loss’ and ‘facial palsy’; however, this association appears to be a novel finding. We review the current literature and discuss otorhinolaryngological presentations of amyloidosis.