Abnormal development of hypoxanthine-guanine phosphoribosyltransferase-deficient CNS neuroblastoma

Connolly, GP, Duley, JA and Stacey, NC (2001) Abnormal development of hypoxanthine-guanine phosphoribosyltransferase-deficient CNS neuroblastoma. Brain Research, 918 1-2: 20-27. doi:10.1016/S0006-8993(01)02909-2

Author Connolly, GP
Duley, JA
Stacey, NC
Title Abnormal development of hypoxanthine-guanine phosphoribosyltransferase-deficient CNS neuroblastoma
Journal name Brain Research   Check publisher's open access policy
ISSN 0006-8993
Publication date 2001
Sub-type Article (original research)
DOI 10.1016/S0006-8993(01)02909-2
Volume 918
Issue 1-2
Start page 20
End page 27
Total pages 8
Place of publication Amsterdam
Publisher Elsevier Science Bv
Language eng
Abstract Lesch-Nyhan syndrome encompasses a host of neurological symptoms, caused by a deficiency of the purine salvage enzyme, hypoxanthine-guanine phosphoribosyltransferase (HGPRT). How the absence of this enzymes activity affects development of the nervous system is unknown. In this study, we examined the ability of N2aTG, a HGPRT-deficient neuroblastoma and its HGPRT-positive counterpart to proliferate and differentiate at various densities. In summary, N2aTG cells proliferated less and differentiated more than N2a cells, with the former cells exhibiting enhanced sensitivity to the effects of low-density culture. Given the homogeneity of this neuroblastoma cell line and its use in studies of neuronal development, the present study indicates that N2aTG cells may prove a suitable in vitro model for the study of non-dopaminergic neuronal development in Lesch-Nyhan syndrome. (C) 2001 Elsevier Science BY All rights reserved.
Keyword Neurosciences
hypoxanthine-guanine phosphoribosyltransferase Lesch-Nyhan syndrome
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Unknown

Document type: Journal Article
Sub-type: Article (original research)
Collection: School of Pharmacy Publications
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Created: Fri, 25 Jan 2008, 15:30:27 EST