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How Huntington's disease affects different neurons | MIT News | Massachusetts Institute of Technology

MIT neuroscientists find two distinct cell populations in the striatum are affected differently by Huntington’s disease. They believe neurodegeneration of one of these populations leads to motor impairments, while damage to the other population, located in a structure called the striosome, may account for the mood disorders that are often see in the early stages of the disease. ....

Ann Graybiel , Myriam Heiman , Richard Faull , Jessicaj Pourian , Manolis Kellis , Robert Buxton , Sergio Sebastian Pineda , Ayano Matsushima , Broad Institute , Artificial Intelligence Laboratory , Nature Communications , Department Of Brain , Mcgovern Institute For Brain Research , Saks Kavanaugh Foundation , National Institutes Of Health , Nancy Lurie Marks Family Foundation , Cognitive Sciences , Picower Institute For , Institute Professor , Mcgovern Institute , Simons Foundation , University Of Auckland , Govern Institute , Brain Research , Picower Institute , National Institutes ,

"The long and the short of Huntington's disease: how the sphingolipid p" by Gabrielle R. Phillips, Jennifer T. Saville et al.

Huntington's disease is a devastating neurodegenerative disorder that onsets in late adulthood as progressive and terminal cognitive, psychiatric and motor deficits. The disease is genetic, triggered by a CAG repeat (polyQ) expansion mutation in the Huntingtin gene and resultant huntingtin protein. Although the mutant huntingtin protein is ubiquitously expressed, the striatum degenerates early and consistently in the disease. The polyQ mutation at the N-terminus of the huntingtin protein alters its natural interactions with neural phospholipids in vitro, suggesting that the specific lipid composition of brain regions could influence their vulnerability to interference by mutant huntingtin; however, this has not yet been demonstrated in vivo. Sphingolipids are critical cell signalling molecules, second messengers and membrane components. Despite evidence of sphingolipid disturbance in Huntington's mouse and cell models, there is limited knowledge of how these lipids are affect ....

Huntingtons Disease , Mass Spectrometry ,

"Phospholipid Profiles Are Selectively Altered in the Putamen and White" by Gabrielle R. Phillips, Sarah E. Hancock et al.

Huntington’s disease (HD) is a genetic, neurodegenerative illness that onsets in late adulthood as a series of progressive and terminal cognitive, motor, and psychiatric deficits. The disease is caused by a polyQ mutation in the Huntingtin gene (HTT), producing a polyglutamine expansion in the Huntingtin protein (HTT). HTT interacts with phospholipids in vitro; however, its interactions are changed when the protein is mutated in HD. Emerging evidence suggests that the susceptibility of brain regions to pathological stimuli is influenced by lipid composition. This study aimed to identify where and how phospholipids are changed in human HD brain tissue. Phospholipids were extracted using a modified MTBE method from the post-mortem brain of 13 advanced-stage HD patients and 13 age-and sex-matched controls. Targeted precursor ion scanning mass spectrometry was used to detect phospholipid species. In the white cortex of HD patients, there was a significantly lower abundance of phosphatidy ....

Huntingtons Disease , White Matter ,

Scientists Identify The Neurons That Process The Outcomes of Actions


Part of weighing up any decision involves considering the consequences that are going to come of it, and scientists have now identified neurons in the brain that seem to encode the outcomes of actions so that they can be properly evaluated. ....

Ann Graybiel , Bernard Bloem , Nature Communications , Massachusetts Institute Of Technology , Massachusetts Institute , Mp Quot ,