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Striatum:
The main components of the
basal ganglia are the striatum.  
The striatum collects information
(signals),  processes the signals
and then sends them through
other parts of the basal ganglia.
 This part of the brain is like the
processor of your personal
computer.
Basal ganglia:
This part of the brain is responsible for processes which includes: attention, short and long term memory,
producing and understanding language, solving problems, making decisions, feelings and thoughts.  
The basal ganglia also controls movement coordination.  Within the brain of an individuals with (JHD/HD)
Huntington's disease, the cells in the Basal ganglia begin to die.  In result, motor skills are affected causing the
uncontrollable movements of the body (chorea).  

It is
very important to understand that HD affects a person emotionally, mentally, and physically, and must be
treated inclusively.  

BDNF: (Brain - derived neurotrophic factor) is a protein related to the "Nerve Growth Factor" (NGF) found in the
brain.  The BDNF helps to keep existing neurons "alive" and to help the growth of new neurons and synapses.
They are found in vital areas of the brain (specifically pertaining to  the striatum in Huntington's disease) for
learning, memory, and thinking.  

Nerves: There are two types of nerves affected by Huntington's disease. "Motor nerves" which send signals from
the central nervous system to the muscular system.

"
Sensory nerves" which receive signals and pass them to the central nervous system.  When HD  attacks the
central nervous system, these nerves are damaged or destroyed.  When this happens, the manifestation of
uncontrollable movements takes place (chorea).
Huntington's disease  (gene found in 1993), is a
hereditary, degenerative, and terminal brain
disease.  Huntington's disease is caused by a
genetic defect on chromosome 4.  Huntington's
disease (HD) or Juvenile Huntington's disease
(JHD), is an autosomal dominant disease. This
means that only one parent must have the
mutated gene for a child to inherit the disease.  

Huntington’s disease slowly diminishes the
affected individual's ability to walk, talk, and
reason. It has been noted that symptoms of
Huntington's disease can begin to show at ages
40-50, however we know that symptoms can
show much earlier in life.  In time, the person
with HD relies completely upon others for their
personal care. HD affects the lives of entire
families - emotionally, psychologically, socially,
and economically.  HD affects males, females,
and knows no ethnic and racial boundaries.  So
many within the international community feels as
if Huntington's disease is like having Parkinson's,
Alzheimer's, and Schizophrenia all at the same
time!

Every child of a person with Huntington's disease
has a 50/50 chance of inheriting the fatal gene.  
Often, the individual with HD might seem to be
(or act) "drunk" to the on-looker, due to the
physical attributes of the disease.  Juvenile HD
(JHD) affects younger individuals. When a youth
has a CAG of 60 or above, and is diagnosed at
age 20 or before, the disease is called Juvenile
Huntington's disease.  Upwards of 5 percent of
known cases of Huntington's disease is the
Juvenile form. Individuals who suffer with JHD
experience the same challenges as individuals
with HD, however the onset of physical and
emotional symptoms happens earlier in life.

*Recent 2013 updated statistics specify that 1 out
of every 7,000 people has HD.*
What is CAG?
When someone has JHD/HD, part of the DNA sequence (called a CAG - cytosine-adenine-guanine) repeats more
times than normal.  Everyone on the planet has a CAG repeat.  The healthy gene repeat is between 10 to 26 times.
 Individuals with a CAG as low as 37 may or may not develop symptoms of the disease. Much more research must
take place with regards to this repeat scale.  Repeats of CAG have been detected over 100 times.  

Graphic (below) showing the excessive repetitions of the cytosine-adenine-guanine (CAG) nucleotide sequence in a
gene from a Huntington's disease patient (bottom) compared to a gene from a person without the
neurodegenerative disorder (top).
Credit: National Institute of General Medical Sciences, National Institutes of Health
- Quest Diagnostics
- http://biology.about.com
- http://ghr.nlm.nih.gov
- https://www.stanford.edu
- http://www.movementdisorders.org
- http://www.wehaveaface.org/JanNolta
- http://www.HDDW.org
- http://www.HDReach.org
*Dr. Peg Nopoulos
George Huntington
described the
Huntington gene
in
1872.
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worldwide who care for
Huntington disease (“HD”)
patients and families and
who are working diligently
to combat this disease.
New research trials for HD
are currently enrolling or
being launched in 2013.  
We  appreciate your time
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difference for Huntington
disease.
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suicide is 2-8 times more
common in HD than the general
population."
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(HD World Congress - 2013)
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