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Current Molecular Medicine
ISSN (Print): 1566-5240
ISSN (Online): 1875-5666
VOLUME: 13
ISSUE: 5
DOI: 10.2174/1566524011313050014      Price:  $58









Disease-Specific iPS Cell Models in Neuroscience

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Author(s): M. Peitz, J. Jungverdorben and O. Brustle
Pages 832-841 (10)
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 gainand 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.
Keywords:
Induced pluripotent stem (iPS) cells, in vitro disease modeling, neurological disorder, neuroscience.
Affiliation:
Institute of Reconstructive Neurobiology, University of Bonn LIFE & BRAIN Center, and LIFE & BRAIN GmbH, Sigmund-Freud Strasse 25, D- 53127 Bonn, Germany.