Why Huntington's Shows Up in Midlife
...until middle age, and the disease is generally fatal 10 to 30 years after onset.
Research at the university's medical school shows that the mutated huntingtin
gene, long known to cause the disease, switches on the JNK3 enzyme produced by neurons, the cells that process and carry signals through the nervous s...
Discovery Sheds Light on Huntington's Disease
...n the corpus striatum -- interacts with the mutant huntingtin
proteins, causing cell death.
The findings expl...t interacted specifically and exclusively with the huntingtin
protein in that part of the brain.
Rhes is foun...nteracted with both healthy and mutant versions of huntingtin
protein, but that it bound much more strongly to m...
Genetic disease treatments and pharmacogenetics: From scientific discovery to medical delivery
...ally, the current results indicate that PPARd (a peroxisome proliferator-activated receptor that is positively modulated by PGC1-a) interacts with the huntingtin
protein, and altered function of PPARd contributes to HD neurodegeneration. If PPARd is involved in this neurological disease, then tractable therapie...
'Intrabody' can mop up mutant protein in Huntington's disease model
...the virus into the brains of mice that make mutant huntingtin
improves their ability to move their limbs, althou... and the cytoplasm," he says. "The intrabody binds huntingtin
proteins with expanded poly-glutamine regions and ...ured cells that make both the intrabody and mutant huntingtin
are able to get rid of the mutant protein faster a...
Breaking down Huntington's disease one protein at a time
...n recent years, there is currently no cure.
The disease begins when the huntingtin
protein falls off HIP1. The vacancy allows another protein, HIPPI, to then ...ity to function normally.
"Until we understand the relationship between huntingtin
protein, HIP1, clathrin and HIPPI -- we are not going to understand what is...
Huntington's disease problem start early
...ollege of Medicine in Houston in a report that appears in the current edition of the journal Neuron.
This research provides evidence of toxicity by huntingtin
(the protein involved in the disease) early during the disease process, said Dr. Juan Botas, associate professor of molecular and human genetics at BC...
Penn talks at ASCB touch on cancer, neurodegenerative diseases, and MD
...ll Biology takes place on December 1-5, 2007 in Washington, D.C.
Some highlights are:
Huntington's Disease: A new role has been discovered for huntingtin
(Htt), the defective protein in Huntington's Disease. Htt is crucial for the movement of packets inside cells that recycle and transport important mol...
A new molecular zip code, and a new drug target for Huntington's disease
...science, Mental Health and Addiction. Even if the huntingtin
protein has been known for almost 20 years, the ca...nces, has been studying the biological role of the huntingtin
protein and the sequences in the protein that tell...Atwal, have discovered a small protein sequence in huntingtin
that allows it to locate to the part of the cell c...
Myelin Associated With Early Evolution of Huntington's Disease
... It affects around 30,000 people in the U.S., with another 150,000 at risk.
While it is known that HD is caused by a mutation in a gene called huntingtin
(Htt), the correct mechanism by which the Htt gene causes or contributes to neuronal cell death and HD symptoms remains uncertain. Bartzokis' research...
Animal Study Found Cure For Huntington Disease
...ered that by preventing the cleavage of the mutant huntingtin
protein responsible for Huntington disease (HD) in...achieved.
‘Ten years ago, we discovered that huntingtin
is cleaved by 'molecular scissors' which led to the hypothesis that cleavage of huntingtin
may play a key role in causing Huntington disease’...
Huntington's disease deciphered
...ollege of Medicine have discovered how the mutated huntingtin
gene acts on the nervous system to create the deva...The researchers were able to show that the mutated huntingtin
gene activates a particular enzyme, called JNK3, w.... They found that at extremely low concentrations, huntingtin
was a potent inhibitor of axonal transport, the sy...
Small molecules might block mutant protein production in Huntington's disease
...f CAG repeats; the effectiveness varied, however, among cells taken from different patients.
Some forms of these compounds left the normal forms of huntingtin
and ataxin-3 proteins undisturbed, but other compounds partly or completely blocked their formation. In some cells, some of the RNA mimics drastically...
New therapeutic strategy could target toxic protein in most patients with Huntington's disease
...fering RNA could reduce production of the damaging huntingtin
protein in nearly half of people with the disease.... disease, which is caused by a mutant form of the huntingtin
is required for healthy nerve cells, but the muta...
Pitt researchers describe molecular '2-step' leading to protein clumps of Huntington's disease
...isease is called huntingtin.
Most people have a huntingtin
protein whose polyglutamine segment contains 20 or...el's, study how polyglutamine expansion alters the huntingtin
In its most recent studies,...out the details of how the aggregation behavior of huntingtin
depends, in a surprisingly intricate way, on the n...
Huntington's disease: catching it early
...ase, called huntingtin, lured an overactive immune response. But since the immune cells that make IL-6 also make huntingtin, it's possible that mutant huntingtin
might wrongly set these cells on attack mode throughout the body. Early intervention strategies to suppress the production of IL-6 might thus stave of...
A protein sequence associated with Huntingtons disease may become life-saving vaccine component
...ngton's disease results from mutations in the protein huntingtin, which contains a sequence of polyglutamine residues (polyQ). In healthy individuals, huntingtin
contains less than 35 glutamines in the polyQ segment. However, in neurons of Huntington's patients, the number of glutamine residues in the sequence ...
Proteasome activator enhances survival of Huntington's disease neuronal model cells
...disease (HD) the disease provoking mutation in the huntingtin
gene eventually causes the cell to build up intran...intraneuronal nuclear protein aggregates of mutant huntingtin
in HD brains indicate dysfunction of the UPS. From...t shows that cellular changes due to expression of huntingtin
protein with longer CAG repeats can reduce the ubi...
MIT research holds promise for Huntington's treatment
...'s disease is caused by misfolded proteins, called huntingtin
proteins, that aggregate and eventually form large...mpounds that prevent or reverse the aggregation of huntingtin
proteins. However, recent evidence suggests that t...ion, thus blocking one of the toxic effects of the huntingtin
The B2 compound also promoted large in...
Old drug shows new promise for Huntington's Disease
...he drug in vivo, on mice bred to express the toxic huntingtin
protein. The mice were given approximately 1 milli...effect may interfere with production of the mutant huntingtin
protein in some way. "But there are still a couple...vitro system in which toxic and non-toxic forms of huntingtin
are made in the same cell. He and his team will th...