A New Ray of Hope for Huntington’s Disease Patients

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gene mutation in huntingtonsIf you are amongst the unfortunate ones who are suffering from or have some near and dear one suffering from a deadly disease called Huntington’s disease, you will be happy to read this. Scientists have gone a step ahead in the treatment of the disease.

What is Huntington’s Disease?

Huntington’s disease is a neurodegenerative disorder caused by a dominant gene’s mutation. Though the mutation occurs in every cell of the body, it is fatal mainly to only two brains cell types. Due to this, symptoms like random, jerky and uncontrollable movements known as chorea are seen in the patients. There are cognitive degeneration symptoms too but they are not seen so predominantly. General restlessness, lack of coordination, slowed saccadic eye movements or uncompleted motions are seen. These symptoms usually occur in middle adult age, i.e. between 35 and 44 years but can occur anytime from infancy. The disease kills about 35,000 people in America alone.

What is the New Research?

The new research has made scientists able to find the origin of the disease in the brain. Researchers at the Semel Institute for Neuroscience and Human Behavior, UCLA, on 28th April have announced this. Because of this, treatments on the disease can get a new improved direction.

The researchers switched the gene to individual parts of brain and examined factors which cause the disease. The research has also shown new targets and courses of treatments which can slow the progress of the disease.

The mutant gene appears in every cell of the body, including all brain cells. Therefore before starting the treatment it is important to know where it originates as told by X. William Yang, who is a professor of psychiatry and biobehavioral sciences at the Semel Institute.

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The mutation is known as Huntington. The disease passes from parent to child.

Scientists have found that a genetic stutter which is a recurring stretch of DNA at one end of the mutated gene causes cell death and brain atrophy which slowly deprives patient of their ability to speak, move, think and eat. At present there is no treatment available for the disease and the patient suffering from an aggressive disease may die within 10 years.

Two Brain Regions Destroyed by the Disease

They are cortex and striatum. Striatum is a cerebral region and is named so because of its striped layers of gray and white matter. More neurons die in this region. However it is not yet found if cortical neurons have a role in the disease, including the malfunction and death of striatal neurons.  

But scientists are surprised to learn that cortical neurons have a major role in starting aspects of the disease in the brain. Shutting down of the mutant gene in cortical region improved the symptoms partially. And shutting it down in both cortical and striatal regions (leaving it intact in other regions of the brain) corrected every symptom, including motor and psychiatric-like impairment and also brain atrophy.

Dr. Yang explains that the mutation brings about lack of communication between cortical and striatal neurons. Therefore by shutting the disease in the cortical region can reestablish the communication and can improve the disease in the striatal region.

The scientists have hoped that because they have now found out the main unanswered question about Huntington’s disease that where it starts, now it will give a definite direction to where the treatment should be targeted – cortical and striatal cells.

So, the scientists will now focus on identifying targets for treatment to normalize the miscommunication and slow down the progress of the disease, and of course, finding out how the mutant gene brings about the degenerative effects in cortical and striatal neurons.

All in all, Huntington’s disease patients have now a ray of hope of treatment.

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