Alzheimer's Disease: The Effects Of Misfolded Protein
371 Words2 Pages
When molecular chaperones fail to protect the protein from improper folding, protein aggregation often occurs and leads to fatal diseases. Improper protein folding and then accumulation cause diseases that include Alzheimer’s and Parkinson’s disease, emphysema, liver damage, sickle cell anemia, cancer and many others.
In Alzheimer’s and Parkinson’s disease, proteins are soluble; however, in these diseases, the proteins conform to insoluble, well-structured fibrillar aggregates. The misfolded proteins accumulate due to the chaperones not keeping up or degrading the damage fast enough (Proteins and Parkinson’s, 2005). Diseases are thought to develop due to the cytotoxic effects of the misfolded proteins and fibrillar aggregates (Ulloa-Aguirre, 2004). The proteins become cytotoxic due to the pore like structures that when formed disrupt the membrane integrity (Valastyan, 2014).…show more content… For example α1-antitrypsin, the protein fails to complete proper folding and is held in the endoplasmic reticulum. The improper folding results in a loss of function for the protein at the desired location and causes a gain of function in the area where it accumulates, instead of being degraded. This specific protein causes emphysema in the location of loss of function and liver damage in the location of gain of function. Liver damage is caused because the protein is synthesized in the endoplasmic reticulum of the hepatocytes and accumulates in this region as well (Valastyan,