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Trial details imported from ClinicalTrials.gov
For full trial details, please see the original record at
https://clinicaltrials.gov/ct2/show/NCT00608881
Registration number
NCT00608881
Ethics application status
Date submitted
4/02/2008
Date registered
6/02/2008
Date last updated
30/03/2016
Titles & IDs
Public title
Coenzyme Q10 in Huntington's Disease (HD)
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Scientific title
Coenzyme Q10 in Huntington's Disease (HD)
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Secondary ID [1]
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5U01NS052592
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Secondary ID [2]
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2CARE 01.00
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Universal Trial Number (UTN)
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Trial acronym
2CARE
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Linked study record
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Health condition
Health condition(s) or problem(s) studied:
Huntington's Disease
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Condition category
Condition code
Intervention/exposure
Study type
Interventional
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Description of intervention(s) / exposure
Treatment: Drugs - coenzyme Q10
Other interventions - placebo
Active Comparator: A - coenzyme Q10 2400 mg/day - Randomized to active treatment (coenzyme Q10 2400 mg/day)
Placebo Comparator: B - Placebo - Randomized to placebo
Treatment: Drugs: coenzyme Q10
4 - 300 mg CoQ chewable wafers taken orally twice a day
Other interventions: placebo
an inactive substance
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Intervention code [1]
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Treatment: Drugs
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Intervention code [2]
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Other interventions
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Comparator / control treatment
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Control group
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Outcomes
Primary outcome [1]
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Joint Rank (Combination of Time to Death (for Subjects Who Died) and Change in Total Functional Capacity Score (TFC) From Baseline to Month 60 (for Subjects Who Survived))
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Assessment method [1]
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The primary outcome variable at the start of the trial was the change in TFC score from baseline to Month 60. The Data and Safety Monitoring Board recommended to the trial leadership that they reconsider how they accommodate missing data from subjects who die in their primary analysis of the change in TFC score. Based on these recommendations, the trial leadership changed the primary analysis to that of a joint rank approach. TFC consists of five ordinally scaled items assessing a person's capacity with: (1) occupation; (2) financial affairs; (3) domestic responsibilities; (4) activities of daily living; and (5) independent living. Total score ranges from zero (worst) to 13 (best).
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Timepoint [1]
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5 years
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Secondary outcome [1]
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Change in Total Functional Capacity (TFC) Score From Baseline to Month 60
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Assessment method [1]
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TFC consists of five ordinally scaled items assessing a person's capacity with: (1) occupation; (2) financial affairs; (3) domestic responsibilities; (4) activities of daily living; and (5) independent living. Total score ranges from zero (worst) to 13 (best).
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Timepoint [1]
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Baseline and Month 60
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Secondary outcome [2]
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Change in Functional Checklist Score From Baseline to Month 60
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Assessment method [2]
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The functional assessment checklist includes 25 questions about common daily tasks. A score of 1 is given for each "yes" reply and a score of 0 is given for each "no" reply (scale range is 0-25). Higher scores indicate better functioning.
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Timepoint [2]
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Baseline and Month 60
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Secondary outcome [3]
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Change in Independence Scale Score From Baseline to Month 60
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Assessment method [3]
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The independence scale assesses independence on a 0 to 100 scale with higher scores indicating better functioning.
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Timepoint [3]
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Baseline and Month 60
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Secondary outcome [4]
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Change in Total Motor Score From Baseline to Month 60
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Assessment method [4]
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The motor section of the Unified Huntington's Disease Rating Scale (UHDRS) assesses motor features of Huntington disease with standardized ratings of oculomotor function, dysarthria, chorea, dystonia, gait, and postural stability. The total motor score is the sum of all the individual motor ratings, with higher scores (124) indicating more severe motor impairment than lower scores. The score ranges from 0 to 124.
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Timepoint [4]
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Baseline and Month 60
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Secondary outcome [5]
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Change in Behavioral Frequency Score From Baseline to Month 60
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Assessment method [5]
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The Unified Huntington's Disease Rating Scale (UHDRS) behavioral subscale assesses frequency and severity of psychiatric-related symptoms, including depressed mood, apathy, low self-esteem/guilt, suicidal thoughts, anxiety, irritable behavior, aggressive behavior, obsessional thinking, compulsive behavior, delusions, and hallucinations. A total score was calculated by summing up all the individual behavioral frequency items (range 0-56) with higher scores representing more severe behavioral impairment.
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Timepoint [5]
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Baseline and Month 60
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Secondary outcome [6]
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Change in Behavioral Frequency x Severity Score From Baseline to Month 60
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Assessment method [6]
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The Unified Huntington's Disease Rating Scale (UHDRS) behavioral subscale assesses frequency and severity of psychiatric-related symptoms, including depressed mood, apathy, low self-esteem/guilt, suicidal thoughts, anxiety, irritable behavior, aggressive behavior, obsessional thinking, compulsive behavior, delusions, and hallucinations. The total score is the sum of the product of the individual behavioral frequency and severity items (range 0-176) with higher scores representing more severe behavioral impairment.
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Timepoint [6]
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Baseline and Month 60
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Secondary outcome [7]
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Change in Symbol Digit Modalities Test (SDMT) From Baseline to Month 60
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Assessment method [7]
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The SDMT assesses attention, visuoperceptual processing, working memory, and cognitive/psychomotor speed. The score is the number of correctly paired abstract symbols and specific numbers in 90 seconds with higher scores indicating better cognitive functioning.
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Timepoint [7]
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Baseline and Month 60
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Secondary outcome [8]
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Change in Verbal Fluency Test From Baseline to Month 60
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Assessment method [8]
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The verbal fluency test is typically considered a measure of executive function. The score is the number of correct words produced across three 1-minute trials.
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Timepoint [8]
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Baseline and Month 60
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Secondary outcome [9]
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Change in Stroop Interference Test - Color Naming From Baseline to Month 60
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Assessment method [9]
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Stroop Interference Test - color naming score is the total number of correct colors identified in 45 seconds and reflects processing speed.
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Timepoint [9]
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Baseline and Month 60
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Secondary outcome [10]
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Change in Stroop Interference Test - Word Reading From Baseline to Month 60
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Assessment method [10]
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Stroop Interference Test - word reading score is the total number of correct words read in 45 seconds and reflects processing speed.
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Timepoint [10]
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Baseline and Month 60
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Secondary outcome [11]
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Change in Stroop Interference Test - Interference From Baseline to Month 60
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Assessment method [11]
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Stroop Interference Test - interference score is the total number of correct items identified in 45 seconds and reflects an executive measure of inhibitory ability.
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Timepoint [11]
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Baseline and Month 60
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Secondary outcome [12]
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Time to a Two-Point Decline in TFC Score or Death
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Assessment method [12]
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TFC consists of five ordinally scaled items assessing a person's capacity with: (1) occupation; (2) financial affairs; (3) domestic responsibilities; (4) activities of daily living; and (5) independent living. Total score ranges from zero (worst) to 13 (best).
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Timepoint [12]
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5 years
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Secondary outcome [13]
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Time to a Three-Point Decline in TFC Score or Death
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Assessment method [13]
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TFC consists of five ordinally scaled items assessing a person's capacity with: (1) occupation; (2) financial affairs; (3) domestic responsibilities; (4) activities of daily living; and (5) independent living. Total score ranges from zero (worst) to 13 (best).
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Timepoint [13]
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5 years
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Secondary outcome [14]
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Number Completing Study at Assigned Dosage Level
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Assessment method [14]
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Timepoint [14]
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5 years
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Eligibility
Key inclusion criteria
To be eligible for enrollment into this study, subjects must meet the following eligibility
criteria within 28 days prior to randomization:
- Subjects must have clinical features of HD and a confirmed family history of HD, OR a
CAG repeat expansion = 36.
- TFC > 9.
- Must be ambulatory and not require skilled nursing care.
- Age = 16 years.
- Women must not be able to become pregnant (e.g., post menopausal, surgically sterile
or using adequate birth control methods for the duration of the study).
- If psychotropic medications are taken (e.g., anxiolytics, hypnotics, benzodiazepines,
antidepressants), they must be at a stable dosage for four weeks prior to
randomization and should be maintained at a constant dosage throughout the study, as
possible. (Note: stable dosing of tetrabenazine is allowable.) Any changes to these
medications mandated by clinical conditions will be systematically recorded and the
subject will be permitted to remain in the trial.
- Able to give informed consent and comply with trial procedures
- Able to take oral medication.
- May be required to identify an informant or caregiver who will be willing and able to
supervise the daily dosing of study medications and to maintain control of study
medications in the home.
- A designated individual will be identified by the subject to participate in the
ongoing consent process should the subject's cognitive capacity to consent become
compromised during participation in the study.
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Minimum age
16
Years
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Maximum age
No limit
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Sex
Both males and females
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Can healthy volunteers participate?
No
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Key exclusion criteria
- History or known sensitivity of intolerability to CoQ.
- Exposure to any investigational drug within 30 days of the Baseline visit.
- Clinical evidence of unstable medical illness in the investigator's judgment.
- Unstable psychiatric illness defined as psychosis (hallucinations or delusions),
untreated major depression or suicidal ideation within 90 days of the Baseline visit.
- Substance (alcohol or drug) abuse within one year of the Baseline visit.
- Women who are pregnant or breastfeeding.
- Use of supplemental coenzyme Q10 within 30 days prior to the Baseline visit
- Clinically serious abnormalities in the screening laboratory studies (Screening
creatinine greater than 2.0, alanine aminotransferase (ALT) or total bilirubin greater
than 3 times the upper limit of normal, absolute neutrophil count of =1000/ul,
platelet concentration of <100,000/ul, hematocrit level of <33 for female or <35 for
male, or coagulation tests > 1.5 time upper limit of normal).
- Known allergy to FD&C yellow #5 or any other ingredient in the study drug (active and
placebo)
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Study design
Purpose of the study
Treatment
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Allocation to intervention
Randomised controlled trial
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Procedure for enrolling a subject and allocating the treatment (allocation concealment procedures)
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Methods used to generate the sequence in which subjects will be randomised (sequence generation)
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Masking / blinding
Blinded (masking used)
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Who is / are masked / blinded?
The people receiving the treatment/s
The people administering the treatment/s
The people analysing the results/data
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Intervention assignment
Parallel
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Other design features
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Phase
Phase 3
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Type of endpoint/s
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Statistical methods / analysis
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Recruitment
Recruitment status
Terminated
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Data analysis
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Reason for early stopping/withdrawal
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Other reasons
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Date of first participant enrolment
Anticipated
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Actual
1/03/2008
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Date of last participant enrolment
Anticipated
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Actual
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Date of last data collection
Anticipated
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Actual
1/05/2015
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Sample size
Target
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Accrual to date
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Final
609
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Recruitment in Australia
Recruitment state(s)
NSW
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Recruitment hospital [1]
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Westmead Hospital, Department of Neurology Level 1, Po Box 533 - Wentworthville
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Recruitment postcode(s) [1]
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2145 - Wentworthville
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Recruitment outside Australia
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United States of America
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State/province [1]
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Alabama
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Arizona
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Arkansas
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California
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United States of America
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Colorado
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United States of America
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Florida
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United States of America
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Georgia
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Idaho
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Illinois
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Indiana
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Iowa
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Kansas
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Maryland
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Massachusetts
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Michigan
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Minnesota
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Missouri
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Nevada
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New Jersey
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New York
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North Carolina
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Ohio
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Pennsylvania
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Tennessee
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Texas
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Canada
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Alberta
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Canada
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British Columbia
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Canada
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Ontario
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Funding & Sponsors
Primary sponsor type
Other
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Name
Massachusetts General Hospital
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Address
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Other collaborator category [1]
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Government body
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Name [1]
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National Institute of Neurological Disorders and Stroke (NINDS)
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Other collaborator category [2]
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Other
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Name [2]
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University of Rochester
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Address [2]
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Ethics approval
Ethics application status
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Summary
Brief summary
The goals of this trial are to determine if coenzyme Q10 is effective in slowing the
worsening symptoms of Huntington's disease and to learn about the safety and acceptability of
long-term coenzyme Q10 use by determining its effects on people with Huntington's disease.
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Trial website
https://clinicaltrials.gov/ct2/show/NCT00608881
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Trial related presentations / publications
Kowall N, Ferrante R, Martin J. Patterns of cell loss in Huntington's disease. Trends in Neurosciences 1987;10:24-29.
Riley D, Lang A. Movement Disorders. In: Bradley W, Daroff R, Fenichel G, eds. Neurology in Clinical Practice. The Neurological Disorders. Boston: Butterworth-Heinemann, 1991: 1563-1601.
Adams P, Falek A, Arnold J. Huntington disease in Georgia: age at onset. Am J Hum Genet. 1988 Nov;43(5):695-704.
Conneally PM. Huntington disease: genetics and epidemiology. Am J Hum Genet. 1984 May;36(3):506-26.
Harper PS. The epidemiology of Huntington's disease. Hum Genet. 1992 Jun;89(4):365-76. doi: 10.1007/BF00194305.
Tanner CM, Goldman SM. Epidemiology of movement disorders. Curr Opin Neurol. 1994 Aug;7(4):340-5. doi: 10.1097/00019052-199408000-00011. No abstract available.
Young AB, Shoulson I, Penney JB, Starosta-Rubinstein S, Gomez F, Travers H, Ramos-Arroyo MA, Snodgrass SR, Bonilla E, Moreno H, et al. Huntington's disease in Venezuela: neurologic features and functional decline. Neurology. 1986 Feb;36(2):244-9. doi: 10.1212/wnl.36.2.244.
Bruyn G. Huntington's chorea: Historical clinical and laboratory synopsis. In: Vinken P, Bruyn G, eds. Handbook of Clinical Neurology. Amsterdam, 1968: 298-378.
Leigh RJ, Newman SA, Folstein SE, Lasker AG, Jensen BA. Abnormal ocular motor control in Huntington's disease. Neurology. 1983 Oct;33(10):1268-75. doi: 10.1212/wnl.33.10.1268.
Caine ED, Hunt RD, Weingartner H, Ebert MH. Huntington's dementia. Clinical and neuropsychological features. Arch Gen Psychiatry. 1978 Mar;35(3):377-84. doi: 10.1001/archpsyc.1978.01770270127013.
Bamford KA, Caine ED, Kido DK, Plassche WM, Shoulson I. Clinical-pathologic correlation in Huntington's disease: a neuropsychological and computed tomography study. Neurology. 1989 Jun;39(6):796-801. doi: 10.1212/wnl.39.6.796.
Sorensen SA, Fenger K. Causes of death in patients with Huntington's disease and in unaffected first degree relatives. J Med Genet. 1992 Dec;29(12):911-4. doi: 10.1136/jmg.29.12.911.
Oliver JE. Huntington's chorea in Northamptonshire. Br J Psychiatry. 1970 Mar;116(532):241-53. doi: 10.1192/bjp.116.532.241. No abstract available.
Greenamyre J, Shoulson I. Huntington's Disease. In: Calne D, ed. Neurodegenerative Diseases. Philadelphia: WB Saunders, 1994: 685-704.
Shoulson I, Fahn S. Huntington disease: clinical care and evaluation. Neurology. 1979 Jan;29(1):1-3. doi: 10.1212/wnl.29.1.1. No abstract available.
Feigin A, Kieburtz K, Bordwell K, Como P, Steinberg K, Sotack J, Zimmerman C, Hickey C, Orme C, Shoulson I. Functional decline in Huntington's disease. Mov Disord. 1995 Mar;10(2):211-4. doi: 10.1002/mds.870100213.
Myers RH, Sax DS, Koroshetz WJ, Mastromauro C, Cupples LA, Kiely DK, Pettengill FK, Bird ED. Factors associated with slow progression in Huntington's disease. Arch Neurol. 1991 Aug;48(8):800-4. doi: 10.1001/archneur.1991.00530200036015.
Penney JB Jr, Young AB, Shoulson I, Starosta-Rubenstein S, Snodgrass SR, Sanchez-Ramos J, Ramos-Arroyo M, Gomez F, Penchaszadeh G, Alvir J, et al. Huntington's disease in Venezuela: 7 years of follow-up on symptomatic and asymptomatic individuals. Mov Disord. 1990;5(2):93-9. doi: 10.1002/mds.870050202.
Young AB, Penney JB, Starosta-Rubinstein S, Markel DS, Berent S, Giordani B, Ehrenkaufer R, Jewett D, Hichwa R. PET scan investigations of Huntington's disease: cerebral metabolic correlates of neurological features and functional decline. Ann Neurol. 1986 Sep;20(3):296-303. doi: 10.1002/ana.410200305.
Kido D, Shoulson I, Manzione J, Harnish P. Measurement of caudate nucleus and putamen atrophy in patients with Huntington's disease. Neuroradiology 1991;33:604-606.
Mazziotta JC. Huntington's disease: studies with structural imaging techniques and positron emission tomography. Semin Neurol. 1989 Dec;9(4):360-9. doi: 10.1055/s-2008-1041346. No abstract available.
Beal MF, Ferrante RJ. Experimental therapeutics in transgenic mouse models of Huntington's disease. Nat Rev Neurosci. 2004 May;5(5):373-84. doi: 10.1038/nrn1386. No abstract available.
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group. Cell. 1993 Mar 26;72(6):971-83. doi: 10.1016/0092-8674(93)90585-e.
Tabrizi SJ, Workman J, Hart PE, Mangiarini L, Mahal A, Bates G, Cooper JM, Schapira AH. Mitochondrial dysfunction and free radical damage in the Huntington R6/2 transgenic mouse. Ann Neurol. 2000 Jan;47(1):80-6. doi: 10.1002/1531-8249(200001)47:13.3.co;2-b.
Cha JH. Transcriptional dysregulation in Huntington's disease. Trends Neurosci. 2000 Sep;23(9):387-92. doi: 10.1016/s0166-2236(00)01609-x.
Ona VO, Li M, Vonsattel JP, Andrews LJ, Khan SQ, Chung WM, Frey AS, Menon AS, Li XJ, Stieg PE, Yuan J, Penney JB, Young AB, Cha JH, Friedlander RM. Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease. Nature. 1999 May 20;399(6733):263-7. doi: 10.1038/20446.
Chen M, Ona VO, Li M, Ferrante RJ, Fink KB, Zhu S, Bian J, Guo L, Farrell LA, Hersch SM, Hobbs W, Vonsattel JP, Cha JH, Friedlander RM. Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease. Nat Med. 2000 Jul;6(7):797-801. doi: 10.1038/77528.
Beal MF, Hyman BT, Koroshetz W. Do defects in mitochondrial energy metabolism underlie the pathology of neurodegenerative diseases? Trends Neurosci. 1993 Apr;16(4):125-31. doi: 10.1016/0166-2236(93)90117-5.
Wellington CL, Ellerby LM, Hackam AS, Margolis RL, Trifiro MA, Singaraja R, McCutcheon K, Salvesen GS, Propp SS, Bromm M, Rowland KJ, Zhang T, Rasper D, Roy S, Thornberry N, Pinsky L, Kakizuka A, Ross CA, Nicholson DW, Bredesen DE, Hayden MR. Caspase cleavage of gene products associated with triplet expansion disorders generates truncated fragments containing the polyglutamine tract. J Biol Chem. 1998 Apr 10;273(15):9158-67. doi: 10.1074/jbc.273.15.9158.
Brouillet E, Hantraye P, Ferrante RJ, Dolan R, Leroy-Willig A, Kowall NW, Beal MF. Chronic mitochondrial energy impairment produces selective striatal degeneration and abnormal choreiform movements in primates. Proc Natl Acad Sci U S A. 1995 Jul 18;92(15):7105-9. doi: 10.1073/pnas.92.15.7105.
Gu M, Gash MT, Mann VM, Javoy-Agid F, Cooper JM, Schapira AH. Mitochondrial defect in Huntington's disease caudate nucleus. Ann Neurol. 1996 Mar;39(3):385-9. doi: 10.1002/ana.410390317.
Koroshetz WJ, Jenkins BG, Rosen BR, Beal MF. Energy metabolism defects in Huntington's disease and effects of coenzyme Q10. Ann Neurol. 1997 Feb;41(2):160-5. doi: 10.1002/ana.410410206.
Sawa A, Wiegand GW, Cooper J, Margolis RL, Sharp AH, Lawler JF Jr, Greenamyre JT, Snyder SH, Ross CA. Increased apoptosis of Huntington disease lymphoblasts associated with repeat length-dependent mitochondrial depolarization. Nat Med. 1999 Oct;5(10):1194-8. doi: 10.1038/13518.
Jenkins BG, Koroshetz WJ, Beal MF, Rosen BR. Evidence for impairment of energy metabolism in vivo in Huntington's disease using localized 1H NMR spectroscopy. Neurology. 1993 Dec;43(12):2689-95. doi: 10.1212/wnl.43.12.2689.
Lodi R, Schapira AH, Manners D, Styles P, Wood NW, Taylor DJ, Warner TT. Abnormal in vivo skeletal muscle energy metabolism in Huntington's disease and dentatorubropallidoluysian atrophy. Ann Neurol. 2000 Jul;48(1):72-6.
Panov AV, Gutekunst CA, Leavitt BR, Hayden MR, Burke JR, Strittmatter WJ, Greenamyre JT. Early mitochondrial calcium defects in Huntington's disease are a direct effect of polyglutamines. Nat Neurosci. 2002 Aug;5(8):731-6. doi: 10.1038/nn884.
Gines S, Seong IS, Fossale E, Ivanova E, Trettel F, Gusella JF, Wheeler VC, Persichetti F, MacDonald ME. Specific progressive cAMP reduction implicates energy deficit in presymptomatic Huntington's disease knock-in mice. Hum Mol Genet. 2003 Mar 1;12(5):497-508. doi: 10.1093/hmg/ddg046.
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Ferrante RJ, Andreassen OA, Jenkins BG, Dedeoglu A, Kuemmerle S, Kubilus JK, Kaddurah-Daouk R, Hersch SM, Beal MF. Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease. J Neurosci. 2000 Jun 15;20(12):4389-97. doi: 10.1523/JNEUROSCI.20-12-04389.2000.
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Summary Results
For IPD and results data, please see
https://clinicaltrials.gov/ct2/show/NCT00608881
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