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Antimalarial Drug Helps to Treat Hereditary Hearing Loss- Research proven

The Researchers from Case Western Reserve University in the US conducted a study and proved the commonly used drug in treating malaria namely artemisinin has shown to prevent genetic and hereditary hearing loss.

Antimalarial drug helps to treat hereditary hearing loss!

The researchers have found that the drug that is used in treatment of malaria can help sensory cells of the inner ear recognize and transport an essential protein to specialized membrane using established pathways within the cell, which will help improve and restore hearing.

The Researchers from Case Western Reserve University in the US conducted a study and proved the commonly used drug in treating malaria namely artemisinin has shown to prevent genetic and hereditary hearing loss.

They have conducted study on the zebra fishes and confirmed the drug has useful potential in preventing hearing loss.

When coming to the mechanism of hearing, The ability of hearing depends on the proteins that reach the outer membrane of the sensory cells in the inner ear, which may be hindered due to certain types of mutations in the protein due to hereditary disorders, thereby preventing it from reaching those membranes.

The sensory cells of the inner ear are covered by hair-like projections, called hair cells, on their surface.
These hair cells have the function to convert vibrations from sounds that reach ear and movements into electrical signals. These electrical signals pass through the nerves and translated in the brain into information for hearing and balance.

Out of various mutations, the mutation in production of the protein called clarin 1- which is seen USHERS syndrome (a condition associated with the hearing loss and visual loss)

This study, has been published in the Proceedings of the National Academy of Sciences (PNAS), found that artemisinin restores cell function of the inner ear( both hearing and balance) in genetically engineered zebrafish that has the same human version of the essential hearing protein.

“We knew mutant protein largely fails to reach the cell membrane, except patients with this mutation are born hearing. This suggested to us that, somehow, at least a fraction of the mutant protein must get to cell membranes in the inner ear,” said Kumar N Alagramam from Case Western Reserve University, senior author of the research.

“If we can understand how the human clarin1 mutant protein is transported to the membrane, then we can exploit that mechanism therapeutically, Using these ‘humanized’ fish models, we were able to study the function of normal clarin1 and, more importantly, the functional consequences of its mutant counterpart,” said Alagramam.

 

During the study  with two drugs: thapsigargin (an anti-cancer drug) and artemisinin (an anti-malarial drug), the researchers have found that the mutant clarin1 protein got trapped in a network of tubules within the cell which helped proteins, including clarin1 to reach their destinations.
Based on this finding, they realized that thereby liberating the mutant protein from the tubules would be the solution they were looking for.

With the drugs the zebrafish larvae did liberated the trapped proteins and have higher clarin1 levels in the membrane; but the drug,  artemisinin was the more effective of the two. Not only did the drug help mutant clarin1 to reach the membrane, hearing and balance functions were better preserved in zebrafish treated with the anti-malarial drug than untreated fish.

drugs the zebrafish larvae

As the survival in the zebrafish depends on normal swim behavior, which in turn depends on balance and the ability to detect water movement( which are hair cell functions in fish). Survival rates in zebrafish expressing the mutant clarin1 jumped from 5% to 45% after artemisinin treatment.

CONCLUSION:

After the research on the humanized fishes, the researchers had found out that arteminin has potential function of helping mutant protein to reach the membrane and thereby helpful in preventing the genetically acquired syndromes like ushers syndrome which is associated with hearing impairment.

 

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