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Huntington’s disease (HD) is a devastating neurodegenerative disorder that is characterized clinically by movement, cognitive, and psychiatric disorders. After illness onset, disability is relentlessly progressive, and the outcome is uniformly fatal. HD is caused by an abnormal CAG triplet repeat expansion in the coding portion (exon 1) of the gene for a protein called huntingtin.1 How the mutation kills neurons is not clear and, as a consequence, there is not yet a way to prevent, slow, or stop the progression of degeneration.
HD runs in families; those at risk and those who are affected know what HD has done to other family members, and they are desperate for a cure. Clinicians who care for HD families often feel powerless and inadequate: temporary symptomatic treatment and palliative support just aren’t enough. We need something better, and we need it now. People with HD are getting worse and dying.
What about replacing the neurons that die in HD? Assuming we know which cells to replace, this is a logical approach. The striatum bears the brunt of the pathology in HD, so this might be a good choice. Conversely, the average brain of someone who has died with HD weighs about 300 grams less than normal, and striatal atrophy can only account for a small fraction of this (∼50 grams). So, although HD pathology is …
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- We need something better, and we need it now: Fetal striatal transplantation in Huntington’s
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