Huntington disease

Common Name(s)

Huntington disease, Huntington's chorea

Huntington disease is a progressive disorder that causes brain cell (neuron) degeneration in certain areas of the brain.  This results in uncontrolled movements, loss of intellectual abilities, and emotional disturbances. Symptoms typically appear between the ages of 35 and 44 years. People with Huntington disease most often live for 15 to 18 years after the condition appears. Huntington disease caused by mutations in the HTT gene and is inherited in an autosomal dominant manner. Each child of a person with Huntington disease has a 50 percent (1 in 2) chance of inheriting the mutation. There is also a less common, early-onset from that begins in childhood or adolescence, called juvenile Huntington disease.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Huntington disease" for support, advocacy or research.

Caring Voice Coalition,Inc,

Caring Voice Coalition empowers patients who live with a life threatening chronic disease through comprehensive outreach programs and services aimed at financial, emotional and educational support. With a steadfast dedication to the organization’s set of core values, our knowledgeable, caring staff maintains our solid reputation as a dependable, responsive organization with a unique, holistic approach to improving the lives of our patients.

Last Updated: 29 Apr 2014

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Hereditary Disease Foundation

The Hereditary Disease Foundation is a non-profit, basic-science organization dedicated to the cure of genetic disease, with a focus on Huntington disease.

Last Updated: 1 Mar 2013

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Huntington's Disease Society of America

We are a national non-profit voluntary health agency dedicated to finding a cure for Huntington's Disease. We provide vital support, information and educational services to improve the lives of those affected by HD, offer resources and guidance for HD families through our national network of volunteer-based chapters and affiliates as well as through our HDSA Centers of Excellence for Family Services, and promote and support research to find a cure for HD.

Last Updated: 11 Oct 2012

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Movement Disorder Society

The Movement Disorder Society (MDS) is an international professional society of clinicians, scientists, and other healthcare professionals, who are interested in Parkinson's disease, related neurodegenerative and neurodevelopmental disorders, hyperkinetic movement disorders, and abnormalities in muscle tone and motor control.

Last Updated: 5 May 2014

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General Support Organizations

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Huntington disease" for support, advocacy or research.

Caring Voice Coalition,Inc,

Caring Voice Coalition empowers patients who live with a life threatening chronic disease through comprehensive outreach programs and services aimed at financial, emotional and educational support. With a steadfast dedication to the organization’s set of core values, our knowledgeable, caring staff maintains our solid reputation as a dependable, responsive organization with a unique, holistic approach to improving the lives of our patients.

http://www.caringvoice.org

Last Updated: 29 Apr 2014

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Hereditary Disease Foundation

The Hereditary Disease Foundation is a non-profit, basic-science organization dedicated to the cure of genetic disease, with a focus on Huntington disease.

http://www.hdfoundation.org

Last Updated: 1 Mar 2013

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Huntington's Disease Society of America

We are a national non-profit voluntary health agency dedicated to finding a cure for Huntington's Disease. We provide vital support, information and educational services to improve the lives of those affected by HD, offer resources and guidance for HD families through our national network of volunteer-based chapters and affiliates as well as through our HDSA Centers of Excellence for Family Services, and promote and support research to find a cure for HD.

http://www.hdsa.org

Last Updated: 11 Oct 2012

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Movement Disorder Society

The Movement Disorder Society (MDS) is an international professional society of clinicians, scientists, and other healthcare professionals, who are interested in Parkinson's disease, related neurodegenerative and neurodevelopmental disorders, hyperkinetic movement disorders, and abnormalities in muscle tone and motor control.

http://www.movementdisorders.org/MDS.htm

Last Updated: 5 May 2014

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General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Huntington disease" returned 369 free, full-text research articles on human participants. First 3 results:

Real-time imaging of glutamate clearance reveals normal striatal uptake in Huntington disease mouse models.
 

Author(s): Matthew P Parsons, Matthieu P Vanni, Cameron L Woodard, Rujun Kang, Timothy H Murphy, Lynn A Raymond

Journal:

 

It has become well accepted that Huntington disease (HD) is associated with impaired glutamate uptake, resulting in a prolonged time-course of extracellular glutamate that contributes to excitotoxicity. However, the data supporting this view come largely from work in synaptosomes, ...

Last Updated: 7 Apr 2016

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The HTT CAG-Expansion Mutation Determines Age at Death but Not Disease Duration in Huntington Disease.
 

Author(s): Jae Whan Keum, Aram Shin, Tammy Gillis, Jayalakshmi Srinidhi Mysore, Kawther Abu Elneel, Diane Lucente, Tiffany Hadzi, Peter Holmans, Lesley Jones, Michael Orth, Seung Kwak, Marcy E MacDonald, James F Gusella, Jong-Min Lee

Journal: Am. J. Hum. Genet.. 2016 Feb;98(2):287-98.

 

Huntington disease (HD) is caused by an expanded HTT CAG repeat that leads in a length-dependent, completely dominant manner to onset of a characteristic movement disorder. HD also displays early mortality, so we tested whether the expanded CAG repeat exerts a dominant influence on ...

Last Updated: 6 Feb 2016

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Treatment with the MAO-A inhibitor clorgyline elevates monoamine neurotransmitter levels and improves affective phenotypes in a mouse model of Huntington disease.
 

Author(s): Marta Garcia-Miralles, Jolene Ooi, Costanza Ferrari Bardile, Liang Juin Tan, Maya George, Chester L Drum, Rachel Yanping Lin, Michael R Hayden, Mahmoud A Pouladi

Journal: Exp. Neurol.. 2016 Apr;278():4-10.

 

Abnormal monoamine oxidase A and B (MAO-A/B) activity and an imbalance in monoamine neurotransmitters have been suggested to underlie the pathobiology of depression, a major psychiatric symptom observed in patients with neurodegenerative diseases, such as Huntington disease (HD). ...

Last Updated: 11 Mar 2016

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Huntington disease" returned 32 free, full-text review articles on human participants. First 3 results:

Huntington Disease in Asia.
 

Author(s): Miao Xu, Zhi-Ying Wu

Journal: Chin. Med. J.. 2015 Jul;128(13):1815-9.

 

The objective was to review the major differences of Huntington disease (HD) in Asian population from those in the Caucasian population.

Last Updated: 26 Jun 2015

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Understanding the Outcomes Measures used in Huntington Disease Pharmacological Trials: A Systematic Review.
 

Author(s): Noelle E Carlozzi, Angela Miciura, Nicholas Migliore, Praveen Dayalu

Journal: J Huntingtons Dis. 2014 ;3(3):233-52.

 

The identification of the gene mutation causing Huntington disease has raised hopes for new treatments to ease symptoms and slow functional decline. As such, there has been a push towards designing efficient pharmacological trials (i.e., drug trials), especially with regard to selecting ...

Last Updated: 10 Oct 2014

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Neuronal Ca(2+) dyshomeostasis in Huntington disease.
 

Author(s): Marta Giacomello, Juan C Oliveros, Jose R Naranjo, Ernesto Carafoli

Journal: Prion. ;7(1):76-84.

 

The expansion of the N-terminal poly-glutamine tract of the huntingtin (Htt) protein is responsible for Huntington disease (HD). A large number of studies have explored the neuronal phenotype of HD, but the molecular aethiology of the disease is still very poorly understood. This ...

Last Updated: 18 Feb 2013

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Last Updated: 2 Mar 2016

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Deep Brain Stimulation (DBS) of the Globus Pallidus (GP) in Huntington's Disease (HD)
 

Status: Recruiting

Condition Summary: Huntington Disease

 

Last Updated: 10 Nov 2016

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Safety Evaluation of Cellavita HD Administered Intravenously in Participants With Huntington's Disease
 

Status: Not yet recruiting

Condition Summary: Huntington Disease

 

Last Updated: 12 Apr 2016

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