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:2/1/2016)... Feb. 1, 2016  Wocket® smart wallet ( www.wocketwallet.com ) announces the ... Joey Fatone . Las Vegas , where ... --> Las Vegas , where Joey appeared at ... The new video ad was filmed at the Consumer Electronics Show (CES2016) ... Wocket booth to meet and greet fans. --> ...
(Date:1/25/2016)... , Jan. 25, 2016  Glencoe Software, the world-leading ... and publication industries, will provide the data management solution ... (NPSC). ... Phenotypic analysis measures the ... organisms, allowing comparisons between states such as health and ...
(Date:1/20/2016)... 20, 2016 A market that just keeps ... from the explosion in genomics knowledge. Learn all about ... A range of dynamic trends are pushing market growth ... medicine - pharmacogenomics - pathogen evolution - next generation ... - greater understanding of the role of genetic material ...
Breaking Biology News(10 mins):
(Date:2/10/2016)... 2016 NX Prenatal Inc., a US ... technology for early warning of adverse pregnancy outcomes, ... study by Dr. Thomas McElrath of ... Fetal Medicine,s (SMFM) annual meeting held in ... 2016.  The presentation reported initial positive top-line results ...
(Date:2/10/2016)... 2016  IsoRay, Inc. (NYSE MKT: ISR), a medical ... radioisotope applications for the treatment of prostate, brain, lung, ... financial results for the second quarter and six months ... --> --> Revenue ... 2016, which ended December 31, 2015, a 12% increase ...
(Date:2/10/2016)... ... February 10, 2016 , ... Benchmark Research, ... the promotion of two long-standing principal investigators (PI) to the roles of Chief ... and Development. , Dr. Laurence Chu, a Benchmark Research PI in the Austin ...
(Date:2/10/2016)... Charlotte, NC (PRWEB) , ... February 10, 2016 ... ... highly anticipated expansion to their comprehensive training and support program, Sonalinkā„¢ remote monitoring. ... Sonablate® HIFU procedures performed on Friday, February 5th, connecting Dr. Samuel Peretsman to ...
Breaking Biology Technology: