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[ New Insight Into The Cellular Defects In Huntington's Disease ]

New Insight Into The Cellular Defects In Huntington's Disease

Huntington's disease is a devastating neurogenerative disorder that causes a progressive loss of functional capacity and reduced life span. It is an inherited condition caused by a mutant HTT gene. Although this has been known for many years, the functions of the normal Htt protein and the mechanisms by which the mutant protein generated from the mutant HTT gene causes disease are not well understood. A team of researchers led by Frà dà ric Saudou, at the Institut Curie, France, has now uncovered a new function for normal Htt protein and determined that this function is disrupted in a mouse model of Huntington disease and in patients with the disorder. Detailed analysis by Saudou and colleagues determined that normal Htt protein regulates the formation of cellular structures known as cilia and that cilia were longer and disorganized in the mouse model of Huntington disease and patients.

Transcriptional Biomarker Identified That Could Help Monitor Huntington's Disease Activity, Evaluate Potential Treatments

Huntington's disease, a devastating genetic disorder that causes degeneration of nerve cells in the brain, affects more than 15, 000 Americans, and at least 150, 000 are at risk of developing the disease. There is no known cure or treatment for the disease - which starts with changes in mood, judgment, memory, and other cognitive functions and inevitably leads to increasing physical disability, dementia and death. In a new research paper published in the Proceedings of the National Academy of Sciences early edition online, researchers identify a transcriptional biomarker that may assist in the monitoring of disease activity and in the evaluation of new medications. The research, which is a collaboration between the laboratory of Clemens Scherzer, MD, in the Center for Neurologic Diseases at Brigham and Women's Hospital (BWH) and the laboratory of Steven Hersch, MD, Ph.

Candidate Therapeutic Target Provided By Link Between Aging And Huntington's Disease

Aging is a major risk factor for the progression of neurodegenerative diseases, including Huntington disease (HD). Morris White and colleagues, at Harvard Medical School, Boston, have now determined that modulating levels of the signaling protein Irs2 changes disease progression in a mouse model of HD. Specifically, increasing Irs2 levels in the brain increased nerve cell damage and reduced lifespan. Conversely, decreasing Irs2 levels reduced nerve cell damage, attenuated symptoms of disease, and increased lifespan. It has been previously shown that reducing Irs2 signaling increases the life span of mice; the data generated by White and colleagues suggests something more specific, that reducing IRS2 signaling could help slow the progression of HD. TITLE: IRS2 increases mitochondrial dysfunction and oxidative stress in a mouse model of Huntington disease View this article at: http://www.

Scientists Discover How Molecular Motors Go Into Energy Save Mode

The transport system inside living cells is a well-oiled machine with tiny protein motors hauling chromosomes, neurotransmitters and other vital cargo around the cell. These molecular motors are responsible for a variety of critical transport jobs, but they are not always on the go. They can put themselves into "energy save mode" to conserve cellular fuel and, as a consequence, control what gets moved around the cell, and when. A new study by Carnegie Mellon University biochemists, published in the Aug. 12 issue of Science, describes how the motors fold in on themselves, or save energy, when their transport services aren't required. According to the researchers, the solution to this molecular puzzle provides new insight into how molecular motor proteins are regulated, and may open new avenues for the treatment of various neurodegenerative diseases, such as Alzheimer's and Huntington's.

Scientist Converts Human Skin Cells Into Functional Brain Cells: Breakthrough Is Likely To Advance Medicine And Human Health

A scientist at the Gladstone Institutes has discovered a novel way to convert human skin cells into brain cells, advancing medicine and human health by offering new hope for regenerative medicine and personalized drug discovery and development. In a paper published online in the scientific journal Cell Stem Cell, Sheng Ding, PhD, reveals efficient and robust methods for transforming adult skin cells into neurons that are capable of transmitting brain signals, marking one of the first documented experiments for transforming an adult human's skin cells into functioning brain cells. "This work could have important ramifications for patients and families who suffer at the hands of neurodegenerative diseases such Alzheimer's, Parkinson's and Huntington's disease, " said Lennart Mucke, MD, who directs neurological research at Gladstone.

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