A DNA Repair Disorder

An article at the website of the National Institute of General Medical Sciences shows a link between faulty DNA repair and Huntington's Disease. As the article entitled 'Study Links Faulty DNA Repair to Huntington’s Disease Onset' reveals oxidative lesions can damage DNA although particular DNA repair enzymes exist enabling organisms to remove such lesions. The process can break down. The following article quote is instructive.

"To see if the oxidative lesions played a role in expansion of the extra DNA segment, the researchers next deleted OGG1, a key enzyme in oxidative lesion repair. Without OGG1, the bulk of the DNA’s oxidative lesions remained untouched, and the inserted segment did not grow at all or it grew far less than in mice carrying a working version of OGG1.

These findings show that while doing its part in removing oxidative lesions, OGG1 triggers a far more damaging effect—the DNA expansion associated with Huntington’s disease."

A DNA segment in those having Huntington's Disease expands. A protein encoded by the relevant gene becomes corrupted and has toxic effects on the brain.

Some of the most revealing experimental results are ones induced by nature. Diseases show what occurs when a biological part malfunctions. Faulty DNA repair engenders genetic disorders that are all too often fatal.