Researchers from South Korea, Sweden, and the United States havecollaborated on a project to restore neuron function to parts ofthe brain damaged by Huntington's disease (HD) by successfully transplanting HD-induced pluripotent stem cells into animal models. Induced pluripotent stem cells (iPSCs) can be geneticallyengineered from human somatic cells such as skin, and can be usedto model numerous human diseases. They may also serve as sources oftransplantable cells that can be used in novel cell therapies. Inthe latter case, the patient provides a sample of his or her ownskin to the laboratory. In the current study, experimental animals with damage to a deepbrain structure called the striatum (an experimental model of HD)exhibited significant behavioral recovery after receivingtransplanted iPS cells. The researchers hope that this approacheventually could be tested in patients for the treatment of HD. "The unique features of the iPSC approach means that thetransplanted cells will be genetically identical to the patient andtherefore no medications that dampen the immune system to preventgraft rejection will be needed," said Jihwan Song, D.Phil.Associate Professor and Director of Laboratory of Developmental &Stem Cell Biology at CHA Stem Cell Institute, CHA University,Seoul, South Korea and co-author of the study. The study, published online this week in Stem Cells , found that transplanted iPSCs initially formed neurons producingGABA, the chief inhibitory neurotransmitter in the mammaliancentral nervous system, which plays a critical role in regulatingneuronal excitability and acts at inhibitory synapses in the brain.GABAergic neurons, located in the striatum, are the cell type mostsusceptible to degeneration in HD. Another key point in the study involves the new disease models forHD presented by this method, allowing researchers to study theunderlying disease process in detail. Being able to control diseasedevelopment from such an early stage, using iPS cells, may provideimportant clues about the very start of disease development in HD.An animal model that closely imitates the real conditions of HDalso opens up new and improved opportunities for drug screening. "Having created a model that mimics HD progression from the initialstages of the disease provides us with a unique experimentalplatform to study Huntington's disease pathology" said PatrikBrundin, M.D., Ph.D., Director of the Center for NeurodegenerativeScience at Van Andel Research Institute (VARI), Head of theNeuronal Survival Unit at Lund University, Sweden, and co-author ofthe study. Huntington's disease (HD) is a neurodegenerative genetic disorderthat affects muscle coordination and leads to cognitive decline andpsychiatric problems. It typically becomes noticeable in mid-adultlife, with symptoms beginning between 35 and 44 years of age. Lifeexpectancy following onset of visual symptoms is about 20 years.The worldwide prevalence of HD is 5-10 cases per 100,000 persons.Key to the disease process is the formation of specific proteinaggregates (essentially abnormal clumps) inside some neurons. Additional References Citations. I am an expert from wire-cabletray.com, while we provides the quality product, such as Sheet Metal Components , Wire Mesh Trays Manufacturer, Basket Cable Tray,and more.
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