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Mice studies & Huntington’s disease


A gene involved in Huntington’s disease has been identified that could counteract the effects of the disease.


Huntington’s disease is caused by a gene mutation which results in the faulty build-up of a protein called huntingtin, and the death of brain cells over time. As yet there is no effective therapy for the disease.


A study, led by EARA member the University of Padua, and including the San Raffaele Scientific Institute, and IRCCS Neuromed Mediterranean Neurological Institute, all Italy, and the University of Vienna, Austria, analysed the stem cells of mice with faulty huntingtin.


They found that a gene (Mtf1) could protect against cell death in mice and zebrafish with the huntingtin mutation.


The team also confirmed their results in stem cells donated by a Huntington’s disease patient.


Senior author Prof Graziano Martello added: “Now we are interested in understanding whether these tools are also suitable for the treatment of [other] neurodegenerative diseases.”


Meanwhile, research at the University of Copenhagen, Denmark, and University of Rochester Medical Center, USA, has found that healthy human glial cells (important for brain function) can be transplanted into the brains of mice to replace aged or diseased cells, commonly seen in brain diseases, such as Huntington’s.

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