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Welcome to the HOPES site!!!

You can return to the main HOPES home page at any time by clicking on the logo in the upper left hand corner of the page.

Our site is divided into five categories (plus a site tools section), which you can access from any page by clicking on the labeled buttons in the top navigation bar. All the pages within a category have the same color navigation bars, so you can always tell where you are within the site. Please click the / buttons to expand/collapse sections:

HD Basics - This is where to start on your first visit to our site. HD Basics is a good way to "get your feet wet" with an introduction to the particulars of HD as well as some background science necessary to understand HD.

The Basics of Huntington's Disease - A basic explanation of the biological basis of Huntington's disease

1. The history, symptoms, and basic mechanism(s) of HD
2. The basic mechanism(s) of HD continued...
3. Video Segments

An Introduction to DNA and Chromosomes - A closer look at what makes up the human genome

1. A closer look at what makes up the human genome...
2. What is DNA? ...Making the single strand.
3. Why is DNA so important?
4. What are complementary strands? ...Making the double helix.
5. What are chromosomes?
6. OK, so what are homologous chromosomes?

An Introduction to Proteins - How proteins are made and what they look like

1. Introduction: What is a protein, and what do proteins do?
2. How are proteins made? (The Central Dogma)
3. What do proteins look like?
4. Example: The huntingtin protein

Juvenile Huntington's Disease - The symptoms and characteristics of juvenile Huntington's disease

1. The symptoms and characteristics of juvenile Huntington's disease.
2. What is juvenile HD?
3. What causes the large CAG repeat numbers seen in juvenile HD cases?
4. How are large repeat numbers related to the increased severity of juvenile HD?
5. How is juvenile HD inherited?
6. What are the early signs of juvenile HD?
7. What symptoms are common to both juvenile HD and adult-onset HD?
8. How are the symptoms of juvenile HD different from those of adult-onset HD?
9. What parts of the brain are affected in juvenile HD?
10. What treatments are recommended specifically for juvenile HD?

The HOPES Timeline - A historical timeline of Huntington's disease

1. Introduction
2. The HOPES Timeline (Flash)
3. The HOPES Timeline: index
4. Scenes 1-5: 16th Century - 1840s
5. Scenes 6-10: 1872 - 1955
6. Scenes 11-15: Late 1950s - 1972
7. Scenes 16-20: 1972 - 1977
8. Scenes 21-25: 1978 - 1981
9. Scenes 26-29: 1983 - 2001
10. Scenes 30-35: Notable People and Places

The HOPES Brain Tutorial - A guided tour of the human brain and its parts

1. A guided tour of the human brain and its parts. (Flash)
2. A guided tour of the human brain and its parts. (non Flash)
3. CNS / Brain Cells / Directions / Protection
4. Cerebral Cortex / Hemispheres / Brain Tissue
5. Lobes
6. Limbic System
7. Basal Ganglia
8. Diencephalon
9. Cerebellum / Reticular Activating System / Brainstem
10. Build A Brain
11. Credits

HOPES For Kids - An Illustrated Introduction to HD, For Kids & Families

1. A Story & Interactive Games for Kids & Families Interested in HD
2. "Bryan's Dad Has Huntington's Disease" (Flash)
3. "Bryan's Dad Has Huntington's Disease", Introduction (non Flash)
4. "Bryan's Dad Has Huntington's Disease", Scenes 1-4 (non Flash)
5. "Bryan's Dad Has Huntington's Disease", Scenes 5-8 (non Flash)
6. "Bryan's Dad Has Huntington's Disease", Scenes 9-12 (non Flash)
7. "Bryan's Dad Has Huntington's Disease", Scenes 13-16 (non Flash)
8. "Bryan's Dad Has Huntington's Disease", Scenes 17-20 (non Flash)
9. "Bryan's Dad Has Huntington's Disease", Scenes 21-23 (non Flash)
10. "Bryan's Dad Has Huntington's Disease", Credits / Acknowledgement / Feedback (non Flash)
11. Interactive games and puzzles
12. Word Search - Game
13. Word Search - Answer Key
14. Crossword Puzzle - Game
15. Crossword Puzzle - Game Clues
16. Crossword Puzzle - Answer Key

Causes of HD - This section concerns those biological mechanisms that are thought to play a role in the development of HD.

The Inheritance of Huntington's Disease - An introductory guide to the genetics of Huntington's disease.

1. An introductory guide to the genetics of Huntington's disease.
2. What is a gene? Which parts of the genetic code correspond to genes?
3. What are alleles? How many alleles can there be for a gene and how many copies does each individual have?
4. How do genes determine physical traits? What does it mean to say that the HD gene is dominant?
5. How are alleles inherited? What are the chances of inheriting the gene?
6. Are my children at risk?
7. There has never been any history of HD in my family...how could the HD allele just suddenly appear?
8. If I have the gene, what does that mean? Will it definitely express itself, and if so, when will this happen?

The Neurobiology of Huntington's Disease - What happens in the brain following the onset of HD?

1. What happens in the brain following the onset of HD?
2. What is a nerve cell?
3. How do nerve impulses work?
4. What parts of the brain are most affected in HD patients?
5. What are the functions of the basal ganglia?
6. How does HD cause striatal and cortical nerve cells to die?

Huntingtin Protein and Protein Aggregation - What Causes the Onset of HD?

1. What Causes the Onset of HD?
2. The Huntington gene
3. What is the function of huntingtin protein?
4. How can the structure of huntingtin be altered?
5. Does mutant huntingtin protein itself kill nerve cells?
6. Altered huntingtin and CREB-binding protein
7. Altered huntingtin and the ubiquitin-proteasome system
8. Why is the altered form of huntingtin not broken down by the cell, as normally occurs with irregular proteins? 
9. What is the process by which protein aggregates interfere with the UPS system?
10. Future challenges for huntingtin research

All About Mutations - What it means to have a mutation and what role mutations play in Huntington's disease.

1. What it means to have a mutation and what role mutations play in Huntington's disease.
2. What are mutations?
3. If an expansion occurs, what is the effect on the child?
4. How do expansions occur?
5. The Unequal Crossing Over Model
6. The Polymerase Slippage Model
7. What is a hairpin and how does it affect mutations?
8. The Hairpin-Mediated Polymerase Slippage Model
9. HD has repeat expansions, but shouldn.t repeat contractions occur as well?
10. Why is it that the longer a parent's CAG repeat region is, the higher the likelihood of expansion?
11. Is expansion more likely to come from the mother or the father?
12. There are many safeguards against mutation. Why don.t these safeguards stop the codon expansion before it becomes harmful?
13. What role does the environment play in mutation?
14. Another theory.
15. A final note

Population Genetics - An examination of the origin and frequency of HD.

1. An examination of the origin and frequency of HD
2. The Genetics of HD
3. The Origin of HD
4. Is it common for new HD alleles to arise?
5. Who is at risk for developing new HD alleles?
6. What is the frequency of HD around the world?
7. What explains the variation in HD prevalence around the world?
8. How does HD affect fertility and reproductive fitness?
9. What has kept the HD allele from being eliminated?
10. Conclusion

Diagnosis - This section gives a summary of many of the symptoms associated with HD.

Genetic Testing - The science and practice of genetic testing for Huntington's disease

1. Introduction
2. How is genetic testing for HD possible?
3. Are there guidelines for genetic testing?
4. Who is at risk for HD?
5. When is an individual tested for HD?
6. Where does genetic testing occur?
7. What are the preliminary steps of the genetic testing process?
8. What does the actual genetic test entail?
9. What are the possible test results?
10. How accurate is the genetic test for HD?
11. Are genetic test results confidential?
12. What are the potential consequences of genetic testing?
13. What if I decide not to be tested?

Behavioral Symptoms - The Behavioral Changes Associated with Huntington's disease

1. Introduction
2. What are some of the behavioral changes that occur in people with HD?
3. What causes the changes in behavior?
4. How do behavior changes vary throughout the course of HD?
5. Will behavior changes occur in every person with HD?
6. Do behavior changes represent the onset of HD in an at-risk individual?
7. Are behavior changes treatable?

Cognitive Symptoms - The Cognitive Changes Associated with Huntington's disease

1. Introduction
2. What are some of the cognitive abilities that may be impaired by HD?
3. What causes the cognitive symptoms of HD?
4. How do the cognitive symptoms change as HD progresses?
5. Do the cognitive symptoms of HD vary from person to person?
6. Are there treatments available for the cognitive symptoms of HD?

Treatments - HD Treatments contains information about drugs and supplements that are being studied as potential HD treatments. This sections also contains summaries of studies that have been done on specific drugs.

Treatment vs. Cure - An Introduction to HD Drugs and Supplements

Disease mechanisms and the treatments that target them

Protein Aggregation - Disease Mechanism I

1. Introduction
2. Geldanamycin
3. RNA Interference (RNAi)
4. Trehalose
5. Cystamine
6. Rapamycin

Inflammation - Disease Mechanism II

1. Introduction
2. HD and Inflammation
3. Inflammatory Mediators
4. Glucocorticoids
5. Nonsteroidal Anti-Inflammatory Drugs (NSAIDS) (Aspirin, Acetaminophen, Ibuprofen)
6. Omega-3 Fatty Acids (EPA (LAX-101), DHA)
7. Minocycline

Abnormalities in Energy Metabolism - Disease Mechanism III

1. Introduction
2. Huntington and Energy Metabolism
3. Creatine
4. Dichloroacetate
5. Nicotinamide
6. Riboflavin
7. Carnitine
8. (TUDCA) Tauroursodeoxycholic acid

Free Radical Damage - Disease Mechanism IV

1. Introduction
2. Free Radicals and Antioxidants
3. Ginkgo Biloba
4. Coenzyme Q10
5. Selenium
6. Lipoic acid
7. Vitamin C

Glutamate Toxicity - Disease Mechanism V

1. Introduction
2. HD and Glutamate
3. Remacemide
4. Riluzole
5. Lamotrigine

Changes in Gene Transcription - Disease Mechanism VI

1. Introduction
2. HDAC Inhibitors - Drugs Able to Affect Gene Transcription

Selective Serotonin Signaling - Disease Mechanism VII

1. About "HD and Serotonin"
2. SSRIs
3. Paroxetine
4. Fluoxetine

Miscellaneous - Disease Mechanism VIII

1. Brain-derived neurotrophic factor
2. Miraxion
3. Tetrabenazine (TBZ)

A simple alphabetical list of drugs and supplements

HOPES Treatments Scorecard

Lifestyle and HD

Diet

Curcumin, the Curry Spice - A look at how curcumin affects cells in Alzheimer's disease and how this research may relate to HD

1. Introduction
2. How Curry Relates to the Epidemiology of Alzheimer's in Humans
3. The Effects of Curcumin On the Cells of Rodents with Alzheimer's
4. How this Alzheimer's Research May Affect Huntington's Disease
5. Uncertainties in How the Animal Research Relates to Humans

Red wine - A look at how resveratrol, a component of red wine, may be used to treat HD

1. Introduction
2. Resveratrol Overview
3. Antioxidant Capabilities
4. Anti-inflammatory Capabilities
5. Details on how resveratrol works
6. Resveratrol and the prevention of neurodegeneration
7. The amount of resveratrol in red wine
8. A word of caution: The dangers of alcohol consumption
9. A final word on resveratrol

Fatty Acids - How fat consumption can influence the course of HD

1. Introduction
2. Saturated vs. Unsaturated Fat
3. Trans fat
4. The relation between fat and nerve cells
5. How fat affects people with HD
6. Getting the right type of unsaturated fat - essential fatty acids.
7. A wrap-up on fatty acids and HD
8. Research on essential fatty acids

Dietary Restriction - A look at how eating less can benefit the body’s cells and combat neurological disease

1. Introduction
2. The Two Different Types of Dietary Restriction
3. An Evolutionary Explanation for Why Dietary Restriction is Effective
4. How HD Kills Nerve Cells and How Dietary Restriction Combats It
5. How Dietary Restriction Reduces Huntingtin Protein Aggregation
6. The Effects of Dietary Restriction on the Onset and Progression of HD
7. The Effects of Dietary Restriction on the Symptoms of HD
8. The Use of Drug Therapy in Place of Dietary Restriction
9. Important Cautionary Note

Stress

1. Introduction
2. A Brief Overview of the Stress Response
3. A Closer Look at the Stress Pathways
4. Harmful Effects of the Stress Response
5. Stress Management

Neuroplasticity

1. Introduction
2. Brain Reorganization: How does neuroplasticity work?
3. Promoting Brain Reorganization: Is it possible to stimulate brain reorganization?
4. Limits of Our Innate Brain Plasticity: Can neuroplasticity repair every brain?
5. Neurons and Neurogenesis: Can new neurons be produced in adult brains?
6. Neuronal Growth Factors: Is there a way to encourage the growth of new neurons?
7. Future Research

Complementary and Alternative Medicine - Treatments for HD

1. Introduction
2. What is Complementary and Alternative Medicine (CAM)?
3. What Types of CAM Therapies are Available?
4. Are CAM Treatments Safe and Effective?
5. Potential CAM Treatments for HD
6. Dietary Supplements

Related Science - This section contains various topics that are related to HD. Some sections deal with areas of science indirectly related to HD, and other sections expand on some studies being conducted on HD.

Info on HD Research

HD in the News

1. Introduction
2. Gene Therapy May Switch Off Huntington's
3. Fasting Forestalls Huntington's Disease in Mice
4. Dye Could Halt Huntington's
5. Amarin Corp. Announces Encouraging Preliminary Results of LAX-101 Pivotal Study in HD
6. Study: Ginkgo Doesn't Aid Memory
7. Study Zeroes in on Causes of Huntington's Disease
8. Huntington's Study Looks Promising
9. HOPES Responds to NY Times Article
10. Stanford Researchers Home in on Huntington's Disease Treatment

Research in Progress

1. Gene Therapy
   1. “Fast and Cheap”: siRNA in Gene Silencing
   2. RNAi: Recognizing the HD Allele
   3. Gene Therapy Successful in HD Rats
2. HD and Lifestyle
   1. Environmental Enrichment Slows Progress of Disease in HD Mice
   2. A Cup of Blueberries a Day Keeps the Doctor Away
   3. Catch o’ the Day
   4. Running for Your Life
   5. Vitamin C Wards Off Colds . . . and HD?
3. Drug Development
   1. Lithium Treatment Protects Nerve Cells in HD Rats
   2. Paxil: For Depression... and HD?
   3. Ever Wonder Why Drugs Fail?
   4. Rapamycin: A New Miracle Drug?
   5. Laser Sheds Light on Mitochondrial Response to Calcium Ions
   6. Hope in a Vial: Possible HD Vaccine
4. Gene Regulation
   1. MicroRNAs and Gene Regulation
   2. Stimulating NeuroD: Turning on a Transcription Factor for Nerve Cell Maintenance
   3. Nuclear Shuttle Proteins and Gene Regulation
   4. Blocking SUMO-1: The End of Early Cell Death?
   5. GRIK2: The Function of a Modifier Gene in HD
   6. Huntingtin on the Job
5. Stem Cell Research News
   1. From Bone Marrow to Brain
6. Cognitive, Emotional, and Rehabilitative Aspects of HD
   1. Changes in Sexual Behavior Accompanying HD
   2. Abnormal Behavior Develops Early in HD Mice
   3. Negative Consequences of Predictive Testing for HD
   4. Texture Modified Diets
7. HD and Other Neurodegenerative Disorders
   1. Neurogenesis in Alzheimer’s Disease: Implications for HD?
   2. Prion-Related Neurodegenerative Disease
   3. Drills and Viruses: Combating Parkinson’s disease
8. Miscellaneous
   1. Kenyon’s Worms: Treating HD and Slowing Down Aging
   2. Osteoporosis: A Risk for HD Women in Long-Term Care Facilities
   3. Meiotic Expansion Mutations
9. Community News Briefs
   1. A Case of Genetic Discrimination?
   2. HD on TV
   3. HD Blog: Discussing the Case of Carol Carr
   4. Carol Carr Granted Parole
   5. New Internet HD Database to be Launched

Research Institutions

1. Introduction
2. Biological Basis of HD
3. HD genetics
4. The Search for Treatments
5. Psychological Studies
6. Clinical Trials

HD Research at Stanford

1. Introduction
2. Ron Kopito, PhD
3. Richard Myers, PhD
4. Lawrence Steinman, MD, PhD

Studying HD

1. Introduction
2. Family-Tree Studies
3. Epidemiological Studies
4. Genetic Studies
5. Linkage Studies
6. Human Postmortem Studies
7. Animal Research
8. Clinical Studies

Stem Cell Research

• Introduction

1. Stem Cell Basics
   1. What is a stem cell?
   2. What are the different kinds of stem cells?
2. Stem Cell Research
   1. What kind of research is being conducted?
   2. What are the advantages and disadvantages of using embryonic stem cells, embryonic germ cells, and adult stem cells for research?
   3. Which are better -- pluripotent stem cells or adult stem cells?
   4. What challenges are researchers facing?
3. Stem Cell Research and Huntington's Disease
   1. What is the potential for using stem cells to treat HD?
   2. What is fetal neural transplantation? What does this have to do with HD and stem cells?
   3. Will stem cell research provide a cure for HD?

Research Frontiers

1. Contents
2. Introduction
3. What’s special about HD research?
4. Conceptualizing HD
5. Approaching HD
6. To hypothesize or not to hypothesize?
7. Accomplishments
8. MacDonald’s Masterminds
9. Debunking the Myths
10. Toward a Treatment: The future of HD research
11. Conclusion

Other info related to HD

Trinucleotide Repeat Disorders

1. Introduction
2. DRPLA (Dentatorubropallidoluysian Atrophy)
3. Huntington's Disease (HD)
4. SBMA (Spinobulbar Muscular Atrophy)
5. SCA1 (Spinocerebellar Ataxia Type 1)
6. SCA2 (Spinocerebellar Ataxia Type 2)
7. SCA3 (Spinocerebellar Ataxia Type 3 or Machado-Joseph Disease)
8. SCA6 (Spinocerebellar Ataxia Type 6)
9. SCA7 (Spinocerebellar Ataxia Type 7)
10. Non-Polyglutamine Diseases

Complications of HD

Comparisons to Other Diseases

1. Introduction
2. Risk Factors
3. Neurobiology: Neuromotor Comparisons
4. Neurobiology: Emotional/Cognitive Comparisons
5. Treatments
6. Prognosis

Kickouts

1. Correlation Without Causality
2. Huntington Gene / HD Allele Clarification
3. The Cerebellum
4. A Note on the Term "Cause"

Site Tools - Here you will find many ways to navigate our site, including a list of all the figures, questions, and glossary entries. You can also search the site, read about HOPES, find links to related sites, or go straight to the newest site additions. You can also access any of these pages by clicking on the image map on the left side of the screen.

Glossary of Science Words - Glossary links that are found in the chapters are collected in the HOPES Glossary, sorted alphabetically for your convenience. There are 694 definitions in the glossary!

1. Glossary: 0-9 ([an error occurred while processing this directive] definitions)
2. Glossary: A ([an error occurred while processing this directive] definitions)
3. Glossary: B ([an error occurred while processing this directive] definitions)
4. Glossary: C ([an error occurred while processing this directive] definitions)
5. Glossary: D ([an error occurred while processing this directive] definitions)
6. Glossary: E ([an error occurred while processing this directive] definitions)
7. Glossary: F ([an error occurred while processing this directive] definitions)
8. Glossary: G ([an error occurred while processing this directive] definitions)
9. Glossary: H ([an error occurred while processing this directive] definitions)
10. Glossary: I ([an error occurred while processing this directive] definitions)
11. Glossary: J ([an error occurred while processing this directive] definitions)
12. Glossary: K ([an error occurred while processing this directive] definitions)
13. Glossary: L ([an error occurred while processing this directive] definitions)
14. Glossary: M ([an error occurred while processing this directive] definitions)
15. Glossary: N ([an error occurred while processing this directive] definitions)
16. Glossary: O ([an error occurred while processing this directive] definitions)
17. Glossary: P ([an error occurred while processing this directive] definitions)
18. Glossary: Q ([an error occurred while processing this directive] definitions)
19. Glossary: R ([an error occurred while processing this directive] definitions)
20. Glossary: S ([an error occurred while processing this directive] definitions)
21. Glossary: T ([an error occurred while processing this directive] definitions)
22. Glossary: U ([an error occurred while processing this directive] definitions)
23. Glossary: V ([an error occurred while processing this directive] definitions)
24. Glossary: W ([an error occurred while processing this directive] definitions)
25. Glossary: X ([an error occurred while processing this directive] definitions)
26. Glossary: Y ([an error occurred while processing this directive] definitions)
27. Glossary: Z ([an error occurred while processing this directive] definitions)

HOPES Site Map - This very page

What's New - Check this page for new and/or updated material.

About HOPES - Read this page for a description of the HOPES group and our mission, and for contact info.

HOPES Site Search - Use this page to search the entire HOPES site for a particular word or phrase on which you might want more information.

External Links - This page contains links to other sites with information related to HD.

Figure Index - This page lists all the figures and tables used in the various chapters.

Kickouts

1. Media Files: Downloading vs. Direct Viewing
2. Media Files: Getting the Appropriate Plug-Ins

Your personal guide to HOPES

Last Modified: 04/12/2007

An educational product of HOPES, not to be used in place of medical care. For more information about HOPES, click on the Logo. To contact HOPES with comments or questions, click here.