Huntington's Disease Severity Slashed by Muscle Relaxant

A muscle relaxant capable of lessening the severity of Huntington's disease (HD) claims a new study.

HD is characterized by ongoing destruction of specific neurons within the brain. It affects a person's ability to walk, talk, and think, leading to involuntary movement and loss of muscle co-ordination.

Huntington's disease
Huntingtons disease is an inherited disease of the nervous system due to the progressive gradual deterioration of the same, initially causing

Progressive damage to medium spiny neurons (MSN) in the brain of a person with HD is responsible for many of the symptoms and is caused by an inherited recessive mutation in the gene "Huntingtin".

The mutated version of this protein leads to abnormal release of calcium from stores within the neurons which in turn disrupts the connections between neurons firing and muscle contractions, and eventually kills the neurons.

According to researchers from the University of Texas Southwestern Medical Center, the RyanR inhibitor Dantrolene is able to reduce the severity of walking and balance problems in a mouse model of HD.

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Ilya Bezprozvanny and his tested Dantrolene, a muscle relaxant that works by stabilizing calcium signalling, and showed that this drug could prevent calcium-dependent toxicity in laboratory grown neurons.

The researchers also found that Dantrolene could prevent destruction of co-ordination, measured by beam walking and footprint patterns, in mice with Huntington's-like disease.

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"One of the features of HD mice is the progressive loss of their NeuN-positive neurons. Dantrolene was not only able to protect muscle co-ordination in mice with HD but also prevented destruction of NeuN positive neurons," Bezprozvanny said.

"Our results suggest that RyanR inhibitors, such as Dantrolene, should be considered as future treatments to slow down the effects of diseases like Huntington's," he added.

The study has been published in the journal Molecular Neurodegeneration.

Source-ANI