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(Ivanhoe Newswire) -- Fruit flies may help lead to a new treatment for children with brain-damaging disorders.
Researchers from Johns Hopkins University genetically modified flies to exhibit symptoms of mucolipidosis type IV (ML4) -- a disease where nerve cells die in the brain and elsewhere. They found the nerve cell death and progression of the disease is linked to a build-up of toxic waste in cells. And they were surprised to discover cell death is delayed by introducing normal blood cells into the flies. This suggests bone marrow transplants may help children affected by this disease and other related disorders.
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Scientists already knew the ML4 disease is caused by loss of the human TRPML1 protein which works in the membranes of the garbage-collector organelles inside of cells - without TRPML, cells build up toxic contents and eventually die.
Essentially, putting TRPML back into blood cells rescued the mutant flies from symptoms of the disease, researcher Craig Montell, Ph.D., Johns Hopkins University School of Medicine, was quoted as saying. After a bit of brainstorming, we came up with the idea that if putting TRPML back into blood cells could do this in flies, maybe it could do so in other animals, including people, using bone marrow transplants to reconstitute blood cells with normal TRPML.
The researchers are now using mice engineered with ML4 to see how they respond to bone marrow transplantation.
SOURCE: Cell, 2008;135:838-851
The article was reported by Ivanhoe.com, which offers Medical Alerts by e-mail every day of the week. To subscribe, click on: http://www.ivanhoe.com/newsalert/.
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