Navigation Links
Developmental disease is recreated in an adult model
Date:6/2/2011

(Cincinnati, OH) An IRSF funded study published today in the journal Science has shown that the childhood disorder Rett syndrome, can be reestablished in adult animals by "switching off" a critical disease causing gene in healthy adult animals. The gene was "switched off" in adult mice by use of a sophisticated genetic trick, resulting in the appearance of behaviors typically seen in Rett syndrome. The leading author Christopher McGraw, MD/PhD student, carried out the study in the laboratory of Dr. Huda Zoghbi, a renowned neuroscientist based at Baylor College of Medicine, and director of the Jan and Dan Duncan Neurological Research Institute at Texas Children's Hospital in Houston TX.

In 1999 Dr. Zoghbi's laboratory made a central discovery, identifying the causative link between mutations in the gene methyl-CpG-binding protein 2 (MeCP2) and Rett syndrome. This work led to other studies showing that MeCP2 protein is critical for the proper functioning of nerve cells during development and into adulthood. In 2007 a further study conducted by Dr. Adrian Bird, at Edinburgh University in the UK, showed the neurological symptoms of Rett syndrome can be reversed by reactivating MeCP2 in an adult mouse where the disease is already established. This work provided a critical proof of concept that symptoms of the disorder may be reversible in humans; however, to-date it was not known whether the early developmental period was important in establishing the course of the disease. This new study argues that early expression of the gene does not protect against the development of symptoms if the disease gene is later inactivated.

Commenting on the study, Dr. Zoghbi said "We did this experiment to see if providing MeCP2 early on in life, during critical periods of brain maturation, would be partially protective from loss of this protein in the adult brain. We were surprised to see that the nervous system had no detectable protection when MeCP2 was lost in adulthood. This affirmed that brain cells must have MeCP2 at all times to function normally."

There have been no effective pharmacological treatments developed to treat the disorder although new therapeutic trials are currently underway. This work suggests that therapies for Rett syndrome may need to be continuously maintained throughout the course of an individual's life.


'/>"/>

Contact: Steve Bajardi
sbajardi@rettsyndrome.org
513-874-3020
International Rett Syndrome Foundation
Source:Eurekalert

Related biology news :

1. Digital zebrafish embryo provides the first complete developmental blueprint of a vertebrate
2. Carnegies Donald Brown receives lifetime achievement award from Society for Developmental Biology
3. Mouse model provides a new tool for investigators of human developmental disorder
4. Specific genetic cause of fetal alcohol-related developmental disorders found
5. Genetic marker linked to problem behaviors in adults with developmental disabilities
6. Developmental delay could stem from nicotinic receptor deletion
7. Chinese scientists discover marker indicating the developmental potential of stem cells
8. Tufts wins NCRR grant for Collaborative Cluster in Genome Structure and Developmental Patterning
9. Developmental problems: Some exist in the genes
10. Fertilizer chemicals linked to animal developmental woes
11. Developmental gene-environment interactions: A model for psychosis
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:6/23/2017)... ITHACA, N.Y. , June 23, 2017 /PRNewswire/ ... leader in dairy research, today announced a new collaboration ... reduce the chances that the global milk supply is ... dairy project, Cornell University has become the newest academic ... Supply Chain, a food safety initiative that includes IBM ...
(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:5/16/2017)... , May 16, 2017   Bridge Patient ... organizations, and MD EMR Systems , an ... partner for GE, have established a partnership to ... product and the GE Centricity™ products, including Centricity ... These new integrations will allow ...
Breaking Biology News(10 mins):
(Date:8/11/2017)... Md. , Aug. 11, 2017 /PRNewswire/ ... a New York Times article regarding the ... billion, according to Kalorama Information.  The article, ... App for That"  used information from ... Patient Monitoring & Telemedicine Market  (Sleep, Diabetes, ...
(Date:8/10/2017)... ... August 09, 2017 , ... ... the agriculture industry reach its ideal customers with the right message. Their effective, ... “As a Midwest company, we realize how crucial the agriculture industry is,” said ...
(Date:8/10/2017)... ... 2017 , ... Okyanos Center for Regenerative Medicine has announced its First Annual ... Freeport, Grand Bahama on September 27, 2017. This daytime event is free to attend, ... Ministry of Health’s National Stem Cell Ethics Committee (NSCEC) and regulations laid out in ...
(Date:8/10/2017)... ... August 10, 2017 , ... ... news outlet had initiated coverage on Next Group Holdings, Inc. and see's significant ... markets geared toward those that cannot engage in traditional banking services. According to ...
Breaking Biology Technology: