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Many of us have heard of the neurological disorder Alzheimer’s disease, which affects 1 in 9 over the age of 65. But fewer are familiar with a rarer form of dementia called Huntington’s disease which affects just 5.7 of every 100,000 people.

May is , so let’s dive in a bit on what this disease is and where we are in treating it.

Discovery and diagnosis of Huntington’s

Huntington’s is an inherited neurological disorder that breaks down nerve cells involved in voluntary movement. The disease was first characterized by George Huntington in his detailing patients’ loss of motor control, jerky movements, personality changes, and cognitive decline. Huntington documented a hereditary pattern for the disease, but it wasn’t until when the Huntington gene (HTT) was molecular mapped to a human chromosome.

Gene regulation

regulates neuronal and glial function in the brain, but an abnormal expansion of glutamine (polyQ) leads to Huntington’s. These abnormal HTT proteins form glue-y plaques in the brain — clumps of the protein with a specific Beta-sheet fold and difficult-to-dissolve (insoluble) structure.

Treating the plaques

Amyloid plaques are a among many neurological diseases (i.e. alpha-synuclein in Parkinson’s, amyloid-beta in Alzheimer’s). These diseases are difficult to treat due to their rigid and insoluble nature and the lack of tools able to disassemble these plaques in cells.

of Huntington’s disease focus on limiting involuntary movements, but unfortunately this is preventative management and not a cure.

Recent for Alzheimer’s targets the amyloid plaque related to the disease, beta-amyloid, and many think similar approaches can be used for other amyloid plaque based diseases including Huntington’s.

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A family history of Alzheimer’s sparks interest in basic research: JBC Herbert Tabor Early Career Investigator Award winner Jenna Lentini shares her work at Discover BMB.

Overcoming missed connections to battle Alzheimer’s: Researchers identify a protein that may allow some people to resist dementia despite plaque accumulation.

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