Huntington's DiseaseNCT01879267
Exercise Effects in Huntington's Disease
A study testing Exercise training in people with Huntington's Disease, run by University of Zurich at 1 site in Switzerland.
Trial summary
Huntington's disease (HD) is an incurable and fatal disorder characterised by progressive degeneration of the basal ganglia and the cerebral cortex. Contrary to earlier thinking, HD is associated with abnormalities in peripheral tissues which might even contribute to brain pathology including muscle wasting… Read more about this study
- Who is it for?
- Ages 30 to 60 years, men only
- Conditions the study is for
- Huntington's Disease
- What is being tested?
- Exercise training
- Type of treatment
- Lifestyle or therapy programme
- Could I receive a placebo?
- No, everyone receives an active treatment
- How many people
- 40 participants
- Where
- 1 siteSwitzerland
- Study dates
- Jan 2012to Jan 2018
- Phase
- Interventional
- Are there results?
- None posted in the registry
Key requirements
Who can take part, as listed by the sponsor. Only the study team can confirm whether you are eligible, so take this list to your doctor.
- Ages 30 to 60 years
- Men only
- No healthy volunteers
- Adult
Who may be able to join
- Male gender
- Genetically verified diagnosis of HD
- Age 30 to 50 years
- Presence of only mild to moderate neurological, cognitive or muscular impairment allowing the subjects to give their written informed consent and to participate in the endurance training (UHDRS 30 or lower)
- For healthy control participants, inclusion criteria are:
- male gender 9
- age 30 to 50 years
- absence of physical or mental illness
Who cannot join
- Female gender
- Advanced neurological, cognitive or muscular impairment related to HD that does not allow patients to participate in the endurance training and/or to give their written informed consent
- Cardiovascular disease or any other medical condition that might not be compatible with endurance training
- CK levels > 300 U/L
- cardiovascular disease
- any other medical condition that might not be compatible with endurance training
- CK levels > 300 U/L.
Trial locations and contacts
1 site in 1 country.
Switzerland1 site
University Hospital Zurich, Division of Neurology
Zurich, Canton of Zurich, 8091
About this study
Huntington's disease (HD) is an incurable and fatal disorder characterised by progressive degeneration of the basal ganglia and the cerebral cortex. Contrary to earlier thinking, HD is associated with abnormalities in peripheral tissues which might even contribute to brain pathology including muscle wasting, mitochondrial abnormalities, and impaired muscle energy metabolism. Mitochondrial impairment and muscle atrophy in human HD patients and murine models of HD are associated with altered expression of PGC-1a, a transcriptional cofactor that seems to regulate many, if not all of the adaptations of muscle fibres to chronic endurance training, and induces improved exercise performance and increased peak oxygen uptake. We aim at investigating whether endurance exercise has the capability of stabilizing and / or reversing PGC-1a dependent alterations of muscle function and structure in HD patients, and whether muscle training ameliorates musculoskeletal and cardiovascular function, as well as motor and cognitive symptoms in HD patients.
Read the detailed description
Huntington disease (HD) is an incurable and fatal disorder that affects muscle function and leads to cognitive decline and dementia. HD was long considered a brain disorder but meanwhile it was shown that HD also affects other tissues such as muscle, leading to muscle wasting. Previous studies suggested that the muscle disorder might be caused by an impaired energy metabolism through mitochondrial dysfunction, which also might also contribute to brain pathology.
In muscle tissue of healthy persons, a protein named PGC 1- α seems to regulate many, if not all of the adaptations of muscle metabolism and mitochondrial biogenesis to chronic endurance training. It was shown that PGC 1- α is reduced in muscle tissue of human HD patients and animal models of HD.
We aim investigating whether endurance exercise has the capability of stabilizing and / or reversing PGC-1α dependent decline of muscle function and structure in HD patients, and whether muscle training ameliorates muscular and cardiovascular function, as well as coordination and cognitive decline in HD. To this end, we will train 20 male HD patients using a 6 months progressive endurance exercise program. In order to compare the size effect of exercise between HD patients and healthy individuals, 20 age-matched healthy males will perform the identical exercise regimen as HD patients. Within one week before the training period starts and within one week after it has ended, we will assess metabolic and functional data. In addition, we will analyze muscle tissue samples for muscle fiber structure, metabolic phenotype and cellular pathology. Finally, gene and protein expression analyses will be performed on muscle tissue extracts to gain insights into the molecular regulation of training adaptations in HD.
- Conditions
- Huntington's Disease
- Being tested
- Exercise training
- Medical subject headings
- Huntington Disease
What participants receive
What participants receive. In a randomised study each person is assigned to one group by chance.
Exercise trainingBehavioural
6 months of exercise training (2 times 30 min per week) starting one week after a 6-months natural course observation period
| Study group | Role | What they receive |
|---|---|---|
| Exercise Training: HD subjects | Other | Endurance exercise for 6 months (30 min per week) starting one week after a 6-months natural course observation Receives: Behavioral: Exercise training |
| Exercise Training: healthy subjects | Other | 6 months of exercise training (2 times 30 min per week) Receives: Behavioral: Exercise training |
What is being measured
The primary outcome is the main question the study is designed to answer.
Change in Unified Huntington's Disease Rating Scale (UHDRS)
Measured: Baseline and 6 months
Timeline
From the start of the study to its completion; the mark is today. Estimated dates are the sponsor’s plan.
- First posted17 Jun 2013 (estimated)
- Study startJan 2012
- Primary completionJan 2018
- Study completionJan 2018
Resources
- Official record on ClinicalTrials.govThe registry entry this page is built from, with its full change history.
- Publications (1)Papers the sponsor has linked to this study.
- Other trials for Huntington's DiseaseStudies of the same condition, open and under way.
- What the trial phases meanHow a treatment moves from first tests in people to approval.
More details
For doctors and researchers: how the study is designed, who runs it, and its registry IDs.
Study design
- Study type
- Interventional
- Phase
- Phase N/A
- Primary purpose
- Treatment
- Allocation
- Non-randomised
- Intervention model
- Parallel groups
- Masking
- None (open label)
- Enrolment
- 40 participants (actual)
Sponsor and investigators
- Lead sponsor
- University of Zurich
- Sponsor type
- University, hospital or other
- Responsible party
- Sponsor
- Investigators
- Hans H Jung, Professor MD, Principal investigator, University Hospital Zurich, Division of Neurology
IDs, dates and publications
- ClinicalTrials.gov ID
- NCT01879267
- Sponsor's protocol ID
- KEK-ZH-Nr. 2009-0119
- Status last verified
- Jun 2018
- First submitted
- 24 May 2012
- Last updated
- 13 Jun 2018
- Results posted
- No
- Data monitoring committee
- Yes
Publications
Mueller SM, Gehrig SM, Petersen JA, Frese S, Mihaylova V, Ligon-Auer M, Khmara N, Nuoffer JM, Schaller A, Lundby C, Toigo M, Jung HH. Effects of endurance training on skeletal muscle mitochondrial function in Huntington disease patients. Orphanet J Rare Dis. 2017 Dec 19;12(1):184. doi: 10.1186/s13023-017-0740-z.
PubMed 29258585
Study at a glance
From the registry record, last updated by the sponsor on 13 Jun 2018. Recruitment status is as the sponsor reported it: confirm with the study team.
- Official title
- Exercise Effects in Huntington's Disease
- ClinicalTrials.gov ID
- NCT01879267
- Acronym / sponsor ID
- KEK-ZH-Nr. 2009-0119
- Conditions
- Huntington's Disease
- Study type
- Interventional
- Recruitment status
- Completed
- Enrolment
- 40 participants (actual)
- Lead sponsor
- University of ZurichUniversity, hospital or other
- Study start
- Jan 2012
- Primary completion
- Jan 2018 (actual)
- Study completion
- Jan 2018 (actual)
- First posted
- 17 Jun 2013 (estimated)
- Results
- Not posted in the source record
Clinical summary
The research question as the sponsor registered it. This describes what the study plans to test, not what it found.
Huntington's disease (HD) is an incurable and fatal disorder characterised by progressive degeneration of the basal ganglia and the cerebral cortex. Contrary to earlier thinking, HD is associated with abnormalities in peripheral tissues which might even contribute to brain pathology including muscle wasting, mitochondrial abnormalities, and impaired muscle energy metabolism. Mitochondrial impairment and muscle atrophy in human HD patients and murine models of HD are associated with altered expression of PGC-1a, a transcriptional cofactor that seems to regulate many, if not all of the adaptations of muscle fibres to chronic endurance training, and induces improved exercise performance and increased peak oxygen uptake. We aim at investigating whether endurance exercise has the capability of stabilizing and / or reversing PGC-1a dependent alterations of muscle function and structure in HD patients, and whether muscle training ameliorates musculoskeletal and cardiovascular function, as well as motor and cognitive symptoms in HD patients.
Detailed description
Huntington disease (HD) is an incurable and fatal disorder that affects muscle function and leads to cognitive decline and dementia. HD was long considered a brain disorder but meanwhile it was shown that HD also affects other tissues such as muscle, leading to muscle wasting. Previous studies suggested that the muscle disorder might be caused by an impaired energy metabolism through mitochondrial dysfunction, which also might also contribute to brain pathology.
In muscle tissue of healthy persons, a protein named PGC 1- α seems to regulate many, if not all of the adaptations of muscle metabolism and mitochondrial biogenesis to chronic endurance training. It was shown that PGC 1- α is reduced in muscle tissue of human HD patients and animal models of HD.
We aim investigating whether endurance exercise has the capability of stabilizing and / or reversing PGC-1α dependent decline of muscle function and structure in HD patients, and whether muscle training ameliorates muscular and cardiovascular function, as well as coordination and cognitive decline in HD. To this end, we will train 20 male HD patients using a 6 months progressive endurance exercise program. In order to compare the size effect of exercise between HD patients and healthy individuals, 20 age-matched healthy males will perform the identical exercise regimen as HD patients. Within one week before the training period starts and within one week after it has ended, we will assess metabolic and functional data. In addition, we will analyze muscle tissue samples for muscle fiber structure, metabolic phenotype and cellular pathology. Finally, gene and protein expression analyses will be performed on muscle tissue extracts to gain insights into the molecular regulation of training adaptations in HD.
Study design
- Primary purpose
- Treatment
- Allocation
- Non-randomised
- Intervention model
- Parallel groups
- Masking
- None (open label)
- Number of arms
- 2
| Arm | Type | Receives |
|---|---|---|
| Exercise Training: HD subjects | Other | Behavioral: Exercise trainingEndurance exercise for 6 months (30 min per week) starting one week after a 6-months natural course observation |
| Exercise Training: healthy subjects | Other | Behavioral: Exercise training6 months of exercise training (2 times 30 min per week) |
Who may qualify
- Ages
- 30 to 60 years
- Sex
- Male only
- Healthy volunteers
- Not accepted
- Age groups
- Adult
Key inclusion criteria (8 listed)
- Male gender
- Genetically verified diagnosis of HD
- Age 30 to 50 years
- Presence of only mild to moderate neurological, cognitive or muscular impairment allowing the subjects to give their written informed consent and to participate in the endurance training (UHDRS 30 or lower)
- male gender 9
Key exclusion criteria (7 listed)
- Female gender
- Advanced neurological, cognitive or muscular impairment related to HD that does not allow patients to participate in the endurance training and/or to give their written informed consent
- Cardiovascular disease or any other medical condition that might not be compatible with endurance training
- CK levels > 300 U/L
- cardiovascular disease
Eligibility is decided by the study team after screening; this overview does not establish that a patient qualifies. Full inclusion and exclusion criteria.
Interventions
| Intervention | Type | Arms |
|---|---|---|
| Exercise training6 months of exercise training (2 times 30 min per week) starting one week after a 6-months natural course observation period | Behavioural | Exercise Training: HD subjects, Exercise Training: healthy subjects |
Primary outcome
Change in Unified Huntington's Disease Rating Scale (UHDRS)
Time frame: Baseline and 6 months
Results and safety
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
Research links
- Official record on ClinicalTrials.govLast updated by the sponsor 13 Jun 2018.
- Derived from this study: PubMed 29258585Mueller SM, Gehrig SM, Petersen JA, Frese S, Mihaylova V, Ligon-Auer M, Khmara N, Nuoffer JM, Schaller A, Lundby C, Toigo M, Jung HH. Effects of endurance training on skeletal muscle mitochondrial function in Huntington disease patients. Orphanet J Rare Dis. 2017 Dec 19;12(1):184. doi: 10.1186/s13023-017-0740-z.
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Referral and contact
AzTrials does not enrol participants or take referrals. Contact the study team or a site listed below; they decide who can take part. This study is not recruiting at the moment.
Hans H Jung, Professor MD principal investigator
University Hospital Zurich, Division of Neurology
1 site in 1 country: see every site and its status.
Questions about this trial
What is this clinical trial studying?
A study testing Exercise training in people with Huntington's Disease, run by University of Zurich at 1 site in Switzerland.
The full registered description is in the About this study section and the official record.
Who may be eligible to take part?
The sponsor lists 8 inclusion and 7 exclusion criteria. Eligibility is decided by the study team after screening, so read the full criteria and contact a listed site.
Is this clinical trial currently recruiting?
The record says: finished, as of the sponsor's last update on 13 Jun 2018. Status can change between updates, so confirm with the study contact before making plans.
Where is the study taking place?
The record lists 1 site in Switzerland. Whether a site is taking part right now should be confirmed with that site. See every site.
What are the possible benefits and risks?
They depend on the study and the treatment, and no study can promise a benefit. The study team explains the known risks during informed consent, before anyone joins; the record itself does not list them.
Are results available for this study?
The study is completed, but results are not available in the source record. That does not mean the treatment did or did not work.
How can a doctor refer a patient or contact the research team?
The record lists no contact. The official record names the sponsor, who can be asked directly. AzTrials does not enrol participants or take referrals.
What does the primary outcome measure?
Change in Unified Huntington's Disease Rating Scale (UHDRS) (time frame: Baseline and 6 months). It is what the study was designed to measure, not a finding.
Where can I read the original study information?
On the ClinicalTrials.gov record for NCT01879267, which also lists 1 linked publication.
Does a listing mean the treatment is approved or proven to work?
No. A listing means the study is registered. It does not show that the treatment is safe, works, or is approved by a regulator.
How often is this information updated?
AzTrials copies changed records from ClinicalTrials.gov every night and reloads the whole registry every week. The sponsor last updated this record on 13 Jun 2018.