title: Disease-specific iPS cell models in neuroscience.
abstract: Neurodegenerative diseases are a heterogeneous group of sporadic or familial disorders of the nervous system that mostly lead to a progressive loss of neural cells. A major challenge in studying the molecular pathomechanisms underlying these disorders is the limited experimental access to disease-affected human nervous system tissue. In addition, considering that the molecular disease initiation occurs years or decades before the symptomatic onset of a medical condition, these tissues mostly reflect only the final phase of the disease. To overcome these limitations, various model systems have been established based on gain and loss-of-function studies in transformed cell lines or transgenic animal models. Although these approaches provide valuable insights into disease mechanisms and development they often lack physiological protein expression levels and a humanized context of molecular interaction partners. The generation of human induced pluripotent stem (hiPS) cells from somatic cells provides access to virtually unlimited numbers of patient-specific cells for modeling neurological disorders in vitro. In this review, we focus on the current progress made in hiPS cell-based modeling of neurodegenerative diseases and discuss recent advances in the quality assessment of hiPS cell lines.
authors: Institute of Reconstructive Neurobiology, University of Bonn LIFE & BRAIN Center, and LIFE & BRAIN GmbH, Sigmund-Freud Strasse 25, D- 53127 Bonn, Germany. brustle@uni-bonn.de
authors: M
authors: M
authors: Peitz
authors: J
authors: J
authors: Jungverdorben
authors: O
authors: O
authors: Brüstle
mesh_terms: D000818:Animals; D018929:Cell Culture Techniques / Q000592:standards; D002478:Cells, Cultured; D006801:Humans; D057026:Induced Pluripotent Stem Cells / Q000502:physiology*; D019636:Neurodegenerative Diseases / Q000473:pathology*; D009488:Neurosciences; D012015:Reference Standards; D012559:Schizophrenia / Q000473:pathology