Huntington's disease normally only begins to cause its tell-tale memory and physical coordination impairments after affected individuals reach the age of 30. The nerve damage that creates these impairments is thought to be partly due to a destructive immune response in the brain. The current study now shows that this inflammatory response starts to brew elsewhere in the body long before it inflicts brain damage.
The British group found high levels of an inflammation-causing protein called IL-6 in the blood of affected individuals more than a decade before they were expected to develop the nervous system symptoms of the disease.
The findings may change scientists' views on the physiological basis of the disease. Before now, it was presumed that brain deposits of the mutant protein that causes the disease, called huntingtin, lured an overactive immune response. But since the immune cells that make IL-6 also make huntingtin, it's possible that mutant huntingtin might wrongly set these cells on attack mode throughout the body. Early intervention strategies to suppress the production of IL-6 might thus stave off brain destruction.
Source: Journal of Experimental Medicine