According to a story from the Washington University School of Medicine in St Louis, scientists from the university used a sample of skin cells from diseased patients and transformed them into the type of brain cell that is affected by Huntington’s disease. These cells are called medium spiny neurons, which die in people with the disease.
Huntington’s disease is an inherited disorder in which the brains cells begin to die. The disease usually begins within 30 to 50 years of age, but can appear at any age. Symptoms may start with changes in personality and mood, followed by more substantial mental problems that eventually lead to dementia. Concurrently, the patient may begin to exhibit chorea, a series of restless, jerking, uncoordinated movement and tremors. Posture begins to change as well, and patients eventually lose the ability to chew, swallow, and speak. At this juncture, there is no cure for Huntington’s disease, although there are treatments available that can reduce the severity of symptoms. The disorder is ultimately fatal; most people die 15 to 20 years after they are diagnosed. To learn more about this condition, click here.
The study illustrated that the converted nerve cells still exhibited the same changes that neurons normally demonstrate in the disease, such as damage to the DNA, dysfunctional mitochondria, and ultimately cell death. However, the scientists were able to reverse the effects by correcting for the genetic malfunctions. The project serves as a way for researchers to understand why medium spiny neurons are affected by the disease. Scientists also hope to use the cell conversion technique in order to test new treatments and drugs that could treat Huntington’s.
Skin cell and brain cells are similar enough that they can be changed into one another without having to revert to the stem cell stage; if the cells had to revert to stem cells, the process would erase many of the age-related effects of Huntington’s disease, which would render them less than useful for study of the disease.
