What is excitotoxicity, and what does it have to do with Hun
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One of the most important things the human brain does is to tell the body what to do and how to feel. An essential amino acid — glutamate — helps the brain perform this vital role, by 'exciting' the neurons or brain cells, which then transmit messages between the brain and the body. A disturbance in this function — either because of excess glutamate or oversensitivity to it — can lead to 'excitotoxicity' or cell death.

Excitotoxicity may contribute to the brain damage occurring acutely after status epilepticus, cerebral ischemia or traumatic brain injury. It may also contribute to chronic neurodegeneration in such disorders as amyotrophic lateral sclerosis (ALS) and Huntington's chorea”.

In short, brain damage after an epileptic seizure or seizures that last for five minutes, ALS, brain stroke and Huntington’s disease are associated with excitotoxity.

What is Huntington’s disease?

Huntington’s disease (HD) is a genetic neurological disorder in which the brain cells (neurons) get progressively damaged. People suffering from Huntington's gradually lose both their mental and physical abilities, and they usually die early. The prevalence of Huntington’s disease in India is lower than in Western countries but higher than in most Asian countries, with more cases reported in the southern states.

Huntington’s disease occurs because of an autosomal dominant mutation - one’s risk for Huntington’s disease goes up by 50% if either parent has it. The gene for Huntington’s disease is present in everybody - it produces a protein known as Huntington that plays a role in the development of neurons. However, in those with Huntington’s disease, this gene is faulty and leads to impaired energy metabolism in brain cells and they produce much less energy than they need.

Ironically, this metabolic defect makes the neurons kick so often and quickly that they start to 'fry'. In medical parlance, this phenomenon is known as excitotoxicity - a portmanteau of the words excited and toxicity.

The disease manifests in the prime years of life - affecting people aged 30 to 50. So far, there’s no cure for the disease.

In late August, scientists at the Salk Institute for Biological Studies in California said they had tested a more advanced system for gene editing than the famous CRISPR on mice. In the future, their gene-editing tool - "intercellular linearized Single homology Arm donor mediated intron-Targeting Integration" or SATI as it is known for short, could help to 'edit' or remove the genes that cause diseases like Huntington's and progeria. The research was published in Cell Research, a peer-reviewed journal.

Source: https://www.firstpost.com/health/what-is-excitotoxicity-and-what-does-it-have-to-do-with-huntingtons-disease-7268231.html
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