Diminuendo -- New mouse model for understanding cause of progressive hearing loss

April 27, 2009

The respective microRNA seed region influences the production of sensory hair cells in the inner ear, both in the mouse and in humans. The findings have been published ahead of print in the current online issue of Nature Genetics. This study represents a major step forward in elucidating the common phenomenon of progressive hearing loss, opening up new avenues for treatment.

Scientists of Helmholtz Zentrum München, led by Professor Martin Hrabé de Angelis, director of the Institute of Experimental Genetics, have developed a new mouse model with a genetic mutant in which a single base of a specific microRNA seed region has been altered. Mice carrying this miR-96 mutation suffer progressive hearing loss as they get older. Moreover, if they carry two of these mutants, their are impaired from birth on.

A number of genes associated with hearing loss were already known. "However, we were very surprised when with our new mouse model we discovered this new class of genes -microRNA - as genetic cause for this clinical picture," explained Dr. Helmut Fuchs, who conceived the idea of this mouse model and who is scientific -technical head of the German Mouse Clinic at Helmholtz Zentrum München.

The new mouse model is called diminuendo, named after the term in music theory meaning "becoming gradually softer". The mice were bred using the ENU method in which the male mice are administered N-ethyl-N-nitrosurea (ENU), thus influencing the DNA of their sperm. Successor generations develop dominant or recessive mutations. Using methods like these, Martin Hrabé de Angelis and his colleagues in the German Mouse Clinic can thus identify mutants that develop diseases similar to human diseases. They made the diminuendo mouse model available to colleagues of the Wellcome Trust Sanger Institute in Cambridge, UK, who - based on specific characterizations - ultimately found the association with the miR-96 mutation.

In Germany alone, around 13 million people have impaired hearing, according to estimates of the German Deaf Association (Deutscher Schwerhörigenbund). There are diverse causes for this, including deafness simply due to old age, hearing loss caused by infections and damage due to chronic noise. However, progressive hearing loss can also have genetic causes.

"We assume that our mouse model will be of far-reaching significance for the development of treatment strategies against genetically caused progressive hearing loss in humans," Dr. Fuchs explained. Colleagues from Spain confirm his assumption. They have already performed first examinations on patients diagnosed with progressive hearing loss. In them the microRNA cluster Mirn96 was mutated in the same seed region as in the mouse model. Now, with the aid of this , the international research consortium hopes to identify factors which are necessary for long-term survival of hair cells and thus to find new approaches for treatment of progressive hearing loss.

More information: Lewis M. et al.: An ENU-induced mutation of miR-96 associated with progressive in mice. Nature Genetics online April 12, 2009, http://dx.doi.org/10.1038/ng.369

Source: German Research Center for Environmental Health


Rank 5 /5 (1 vote)
Related Stories
Relevant PhysicsForums posts

More news stories

Complex wiring of the nervous system may rely on a just a handful of genes and proteins

Researchers at the Salk Institute have discovered a startling feature of early brain development that helps to explain how complex neuron wiring patterns are programmed using just a handful of critical genes. ...

Medicine & Health / Research

created 4 hours ago | popularity 5 / 5 (7) | comments 0 | with audio podcast

Both maternal and paternal age linked to autism

Older maternal and paternal age are jointly associated with having a child with autism, according to a recently published study led by researchers at The University of Texas Health Science Center at Houston (UTHealth).

Medicine & Health / Psychology & Psychiatry

created 9 hours ago | popularity 5 / 5 (1) | comments 0 | with audio podcast

New understanding of DNA repair could eventually lead to cancer therapy

A research group in the Faculty of Medicine & Dentistry at the University of Alberta is hoping its latest discovery could one day be used to develop new therapies that target certain types of cancers.

Medicine & Health / Cancer

created 8 hours ago | popularity 4.8 / 5 (4) | comments 0 | with audio podcast

Curry spice component may help slow prostate tumor growth

Curcumin, an active component of the Indian curry spice turmeric, may help slow down tumor growth in castration-resistant prostate cancer patients on androgen deprivation therapy (ADT), a study from researchers ...

Medicine & Health / Cancer

created 10 hours ago | popularity 4.5 / 5 (6) | comments 0 | with audio podcast

Human cognitive performance suffers following natural disasters, researchers find

Not surprisingly, victims of a natural disaster can experience stress and anxiety, but a new study indicates that it might also cause them to make more errors - some serious - in their daily lives. In their upcoming Human Fa ...

Medicine & Health / Psychology & Psychiatry

created 6 hours ago | popularity 4 / 5 (1) | comments 0


Google users warned of threat to smartphone wallets

Users of Google smartphone wallets were being warned on Friday that there is a way to crack pass codes intended to thwart thieves from going on illicit shopping sprees.

Anonymous knocks CIA website offline (Update)

The website of the Central Intelligence Agency was inaccessible on Friday after the hacker group Anonymous claimed to have knocked it offline.

New error-correcting codes guarantee the fastest possible rate of data transmission

Error-correcting codes are one of the triumphs of the digital age. They’re a way of encoding information so that it can be transmitted across a communication channel — such as an optical fiber o ...

Putting the squeeze on planets outside our solar system

(PhysOrg.com) -- Using high-powered lasers, scientists at Lawrence Livermore National Laboratory and collaborators discovered that molten magnesium silicate undergoes a phase change in the liquid state, abruptly ...

The power of estrogen -- male snakes attract other males

A new study has shown that boosting the estrogen levels of male garter snakes causes them to secrete the same pheromones that females use to attract suitors, and turned the males into just about the sexiest ...

NASA sees wide-eyed cyclone Jasmine

Cyclone Jasmine's eye has opened wider on NASA satellite imagery, as it moves through the Southern Pacific Ocean.