ABSTRACT
Huntington's disease (HD) is a progressive neurodegenerative disorder that affects between 5 and 10 out of 100,000 individuals throughout the world. A promising complementary approach is the use of human embryonic stem cells and induced pluripotent stem cells derived from HD patient cells to model HD. Attempts to model HD initially focused on transgenic animal models, immortalized human cell lines, and postmortem tissue from HD patients. Microglia originate from hematopoietic stem cells in the bone marrow, and during development, they cross into the brain through the blood brain barrier where they reside and proliferate upon immune stimuli. Striatal Medium spiny neurons are the predominant cell type that dies in HD; however, a number of other cell types are also affected. Along with changes in organ function, the peripheral innate immune system is altered in HD and numerous inflammatory cytokines are detected.
