Describe what is meant by a "conformational disease" and give an example. Why do you think these diseases exhibit late age of onset?

Question

What will be an ideal response?

Answer 1

Diseases resulting from the death of cells due to accumulation of misfolded proteins are classified as conformational diseases. Several human protein conformational diseases are known, and they are often neurodegenerative disorders and dementias. For example, Alzheimer disease is produced by the accumulation of misfolded β-amyloid protein that leads to the destruction of certain brain cells. A familial form of Parkinson disease produced by the accumulation of misfolded α-synuclein protein also results in the destruction of certain brain cells. Similarly, Huntington disease is produced by the destruction of neurons due to the accumulation of misfolded huntington protein. Each of these diseases has a late age of onset that is due to gradual cell death caused by the presence of abnormal aggregates of misfolded protein. These diseases are neuronal because that is where the genes encoding the proteins are expressed.