Health Observance

Huntington’s disease: a rare cousin of Alzheimer’s

A snapshot to raise awareness
Chloe Kirk
May 14, 2023

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 Huntington’s Disease Awareness Month, 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 1872 paper 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 1983 when the Huntington gene (HTT) was molecular mapped to a human chromosome.

Gene regulation

HTT 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 characteristic 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.

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

Recent drug approval 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.

Related stories

Huntingtin through a multiomic lens: A study shows that the mutant protein that causes Huntington’s disease can alter the binding properties of another protein, perhaps accounting for some of the mutation’s far-flung cellular effects.

A very delicate balance: Could blocking lysosomal gatekeeper PIKfyve slow neurodegeneration?

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.

Reimagining drugs for rare brain disorder: Researchers develop new pipeline to screen large number of existing compounds to find a therapy for an ataxia.

Neurodegenerative disease linked to microtubules: A team at McGill University reports a new role for sacsin, the protein mutated in a rare hereditary ataxia.

Enjoy reading ASBMB Today?

Become a member to receive the print edition four times a year and the digital edition monthly.

Learn more
Chloe Kirk

Chloe Kirk is working toward her Ph.D. in biochemistry and molecular biology at the University of Miami. Her interests are science research, communication and outreach.

Get the latest from ASBMB Today

Enter your email address, and we’ll send you a weekly email with recent articles, interviews and more.

Latest in Science

Science highlights or most popular articles

How signals shape DNA via gene regulation
Journal News

How signals shape DNA via gene regulation

Aug. 19, 2025

A new chromatin isolation technique reveals how signaling pathways reshape DNA-bound proteins, offering insight into potential targets for precision therapies. Read more about this recent MCP paper.

A game changer in cancer kinase target profiling
Journal News

A game changer in cancer kinase target profiling

Aug. 19, 2025

A new phosphonate-tagging method improves kinase inhibitor profiling, revealing off-target effects and paving the way for safer, more precise cancer therapies tailored to individual patients. Read more about this recent MCP paper.

How scientists identified a new neuromuscular disease
Feature

How scientists identified a new neuromuscular disease

Aug. 14, 2025

NIH researchers discover Morimoto–Ryu–Malicdan syndrome, after finding shared symptoms and RFC4 gene variants in nine patients, offering hope for faster diagnosis and future treatments.

Unraveling cancer’s spaghetti proteins
Profile

Unraveling cancer’s spaghetti proteins

Aug. 13, 2025

MOSAIC scholar Katie Dunleavy investigates how Aurora kinase A shields oncogene c-MYC from degradation, using cutting-edge techniques to uncover new strategies targeting “undruggable” molecules.

How HCMV hijacks host cells — and beyond
Profile

How HCMV hijacks host cells — and beyond

Aug. 12, 2025

Ileana Cristea, an ASBMB Breakthroughs webinar speaker, presented her research on how viruses reprogram cell structure and metabolism to enhance infection and how these mechanisms might link viral infections to cancer and other diseases.

Understanding the lipid link to gene expression in the nucleus
Profile

Understanding the lipid link to gene expression in the nucleus

Aug. 11, 2025

Ray Blind, an ASBMB Breakthroughs speaker, presented his research on how lipids and sugars in the cell nucleus are involved in signaling and gene expression and how these pathways could be targeted to identify therapeutics for diseases like cancer.