Neural progenitor cells from an adult patient with fragile X syndrome

• Main Authors: Nethercott Hubert E (Author), Greco Claudia M (Author), Tassone Flora (Author), Schwartz Philip H (Author), Ziaeian Boback (Author), Hagerman Randi J (Author), Hagerman Paul J (Author)
• Format: Book
• Published: BMC, 2005-01-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.

Summary

<p>Abstract</p> <p>Background</p> <p>Currently, there is no adequate animal model to study the detailed molecular biochemistry of fragile X syndrome, the leading heritable form of mental impairment. In this study, we sought to establish the use of immature neural cells derived from adult tissues as a novel model of fragile X syndrome that could be used to more fully understand the pathology of this neurogenetic disease.</p> <p>Methods</p> <p>By modifying published methods for the harvest of neural progenitor cells from the post-mortem human brain, neural cells were successfully harvested and grown from post-mortem brain tissue of a 25-year-old adult male with fragile X syndrome, and from brain tissue of a patient with no neurological disease.</p> <p>Results</p> <p>The cultured fragile X cells displayed many of the characteristics of neural progenitor cells, including nestin and CD133 expression, as well as the biochemical hallmarks of fragile X syndrome, including CGG repeat expansion and a lack of FMRP expression.</p> <p>Conclusion</p> <p>The successful production of neural cells from an individual with fragile X syndrome opens a new avenue for the scientific study of the molecular basis of this disorder, as well as an approach for studying the efficacy of new therapeutic agents.</p>

Item Description

10.1186/1471-2350-6-2
1471-2350