Navigation Links
Diminuendo -- New mouse model for understanding cause of progressive hearing loss
Date:4/27/2009

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 Mnchen, led by Professor Martin Hrab 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 sensory hair cells 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 Mnchen.

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 Hrab 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 Schwerhrigenbund). 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 mouse model, 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.


'/>"/>

Contact: Sven Winkler
sven.winkler@helmholtz-muenchen.de
0049-893-187-3946
Helmholtz Zentrum Mnchen - German Research Center for Environmental Health
Source:Eurekalert

Related biology news :

1. Mouse model provides a new tool for investigators of human developmental disorder
2. CSHL team develops mouse models of leukemia that predict response to chemotherapy
3. Brain building: Study shows brain growth tied to cell division in mouse embryos
4. A worm-and-mouse tale: B cells deserve more respect
5. Salk researchers develop novel glioblastoma mouse model
6. Peering inside the skull of a mouse to solve meningitis mystery
7. Multiple sclerosis research charges ahead with new mouse model of disease
8. Anti-cancer drug prevents, reverses cardiovascular damage in mouse model of premature aging disorder
9. Anything but modest: The mouse continues to contribute to humankind
10. Let the cat keep chasing the mouse
11. Mouse model developed at UT Southwestern mimics hyperglycemia, aids in diabetes research
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:5/23/2017)... , May 23, 2017  Hunova, the first robotic gym for the ... been officially launched in Genoa, Italy . The first ... and the USA . The technology was developed ... market by the IIT spin-off Movendo Technology thanks to a 10 million ... News Release, please click: ...
(Date:4/24/2017)... April 24, 2017 Janice Kephart ... with  Identity Strategy Partners, LLP (IdSP) , today ... without President Trump,s March 6, 2017 Executive ... , refugee vetting can be instilled with greater confidence, ... now, all refugee applications are suspended by until ...
(Date:4/13/2017)... MONICA, Calif. , April 13, 2017 ... New York will feature emerging and evolving ... Summits. Both Innovation Summits will run alongside the expo ... of speaker sessions, panels and demonstrations focused on trending ... coast,s largest advanced design and manufacturing event will take ...
Breaking Biology News(10 mins):
(Date:8/16/2017)... ... 2017 , ... Today, 3Bar Biologics Inc ., the ... funding from an impressive group of investors, including Rev1 Ventures, Maumee Ventures, Ohio ... investment, 3Bar is broadening availability of its groundbreaking offering that uses naturally occurring ...
(Date:8/15/2017)... After spending the past two years building a state-of-the-art ... GeneFo now offers this platform to healthcare stakeholders (hospitals, foundations, biopharma ... and data collection vis a vis their members, under their own ... of this offer. ... ...
(Date:8/15/2017)... ... 2017 , ... Any expert in stem cell research or stem cell medicine ... half a century. Despite their essential roles in human health and regenerative medicine, ... tags developed for this purpose also tag other, more abundant, non-stem tissue cells ( ...
(Date:8/15/2017)... ... August 15, 2017 , ... JULABO USA introduces ... the new website makes it easy to navigate through the site whether you’re ... find detailed product information, educational industry content and visit the company’s social media ...
Breaking Biology Technology: