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Scientists find clue to neurodegeneration process.

TOKYO, Aug. 28 Kyodo

A team of Japanese researchers has discovered clues as to how some neurodegenerative diseases occur, possibly paving the way for effective medical treatment of nervous disorders such as Huntington's disease and spinal and bulbar muscular atrophy.

The scientists found that, in at least eight serious hereditary neurodegenerative illnesses, a substance produced by genetic parts common to causal genes enters the nuclei of nerve cells in the brain and suppresses their functions.

They have also successfully recovered the function of the nerve cells in in-vitro experiments, according to a report of their findings, to be published in the September issue of U.S. medical magazine Nature Genetics due out Monday.

The team comprises Shoji Tsuji, a professor at the Department of Neurology of Niigata University's Brain Research Institute, Takayoshi Shimohata, a graduate student at the institute, University of Tsukuba lecturer Toshihiro Nakajima, and 25 others.

The parts common to causal genes are where repeats of three types of chlorine -- cytosine (C), adenine (A) and guanine (G) -- expand abnormally to form CAG chains. These parts produce polyglutamine, a highly toxic substance believed to kill cerebral nerve cells.

While previous research has shown that polyglutamine enters the nuclei of nerve cells, the latest findings indicate that the substance binds with a protein called TAF-II-130, one of the factors involved in genetic transcriptional activation, thereby suppressing the transcription process.

In-vitro tests conducted by the group also showed that the functions of the nerve cells were fully recovered after supplementing the cells with TAF-II-130.

''This time, we not only discovered the pathogenic mechanism of the diseases, but also started to see that we can reverse the degeneration of nerves -- something that had previously been thought impossible,'' Tsuji said.

''Of course, we cannot apply the methods to humans immediately, but there are many possibilities in terms of prevention. It may soon become possible for doctors to diagnose conditions and administer treatment at early stages of the disorders,'' he added.
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Publication:Japan Science Scan
Date:Sep 4, 2000
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