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Huntington disease begins to take hold early on
April 17, 2009A global analysis of brain proteins over a 10-week period in a mouse model of Huntington Disease has revealed some new insights into this complex neurodegenerative disorder. For example, profound changes (comparable to those seen in late-stage HD) actually occur well before any disease symptoms show up, and most of the changes are confined to a specific stage during disease progression. These findings should aid in determining the optimal times for therapies that aim to treat or cure this disease.
While HD (which is brought on by mutations in the gene for Huntingtin has been studied extensively at the cellular level, much of the work has been focused on late-stage disease when the various symptoms (declines in both motor coordination and cognitive ability) have already manifested. But since HD is an inherited condition, changes likely occur much earlier, and to get a better sense of disease progression, Claus Zabel and colleagues used proteomics to analyze the brains of HD mice at 2, 4, 6, 8, and 12 weeks of age, a period that covers absence of any disease-related phenotypes to the pronounced disease state.
Unexpectedly, they found a large number of protein alterations (almost 6% of the total) as early as 2 weeks of age; a significant portion of these changes contributed to an increase in glucose metabolism, which corresponds to the weight loss that occurs early during HD progression. As the disease progressed over 10 weeks, though, the affected proteins kept changing. In fact, about 70% of observed changes were confined to one of the five time points examined and no proteins were similarly altered in all 5 stages.
Therefore this study, appearing in the April issue of Molecular and Cellular Proteomics, argues against an HD model in which there is a gradual increase in the number and magnitude of protein changes and instead leans toward a more dynamic pathology. Zabel and colleagues suggest that these early changes affect late stage disease by irreversibly changing the biochemical activity in the mouse brain.
American Society for Biochemistry and Molecular Biology
Therefore this study, appearing in the April issue of Molecular and Cellular Proteomics, argues against an HD model in which there is a gradual increase in the number and magnitude of protein changes and instead leans toward a more dynamic pathology. Zabel and colleagues suggest that these early changes affect late stage disease by irreversibly changing the biochemical activity in the mouse brain.
American Society for Biochemistry and Molecular Biology
Neurodegenerative Current Events and Neurodegenerative News RSSCaltech researchers show efficacy of gene therapy in mouse models of Huntington's disease
Researchers at the California Institute of Technology (Caltech) have shown that a highly specific intrabody (an antibody fragment that works against a target inside a cell) is capable of stalling the development of Huntington's disease in a variety of mouse models.
Inhibitor of heat shock protein is a potential anticancer drug, Penn study finds
Like yoga for office drones, cells do have coping strategies for stress. Heat, lack of nutrients, oxygen radicals - all can wreak havoc on the delicate internal components of a cell, potentially damaging it beyond repair.
Widely used cholesterol-lowering drug may prevent progression
Simvastatin, a commonly used, cholesterol-lowering drug, may prevent Parkinson's disease from progressing further. Neurological researchers at Rush University Medical Center conducted a study examining the use of the FDA-approved medication in mice with Parkinson's disease and found that the drug successfully reverses the biochemical, cellular and anatomical changes caused by the disease.
Member of NFL Hall of Fame diagnosed with degenerative brain disease
The Center for the Study of Traumatic Encephalopathy (CSTE) at Boston University School of Medicine (BUSM) announced today that a recently deceased member of the NFL Hall of Fame suffered from the degenerative brain disease Chronic Traumatic Encephalopathy (CTE) when he died, becoming the 10th former NFL player diagnosed with the disease.
Trembling hands and molecular handshakes
Fragile X tremor/ataxia syndrome (FXTAS) is a recently recognized condition, which is actually one of the most prevalent heritable neurodegenerative diseases.
Neurologists Investigate Possible New Underlying Cause of MS
Neurologists at the University at Buffalo are beginning a research study that could overturn the prevailing wisdom on the cause of multiple sclerosis (MS).
First former college football player diagnosed with CTE
The Center for the Study of Traumatic Encephalopathy (CSTE) at Boston University School of Medicine (BUSM) announced today that a deceased former college football player who died at age 42 was already suffering from the degenerative brain disease, Chronic Traumatic Encephalopathy (CTE).
Manipulating Brain Inflammation May Help Clear Brain of Amyloid Plaques, Mayo Clinic Researchers Say
In a surprising reversal of long-standing scientific belief, researchers at the Mayo Clinic campus in Florida have discovered that inflammation in the brain is not the trigger that leads to buildup of amyloid deposits and development of Alzheimer's disease.
Compound shows potential for slowing progression of ALS
A chemical cousin of a drug currently used to treat sepsis dramatically slows the progression of amyotrophic lateral sclerosis, better known as ALS or Lou Gehrig's disease, in mice.
Angiochem crosses BBB, shows safety, efficacy in phase 1/2 brain cancer studies
Angiochem, Inc. a clinical-stage biotechnology company developing drugs that are uniquely capable of crossing the blood-brain barrier to treat brain diseases, announced today that its lead drug candidate, ANG1005, has demonstrated a favorable safety and efficacy profile in more than 100 patients with brain cancer from two separate Phase 1 /2 clinical studies in patients with progressive gliomas, including recurrent glioblastoma, and in patients with progressive brain metastases.
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