Hypoxanthine-guanine phosphoribosyltransferase-deficiency produces aberrant neurite outgrowth of rodent neuroblastoma used to model the neurological disorder Lesch Nyhan syndrome
Lesch Nyhan syndrome (LNS) manifests in bizarre and horrific neurological symptoms, the primary cause being a deficiency of the purine salvage enzyme, hypoxanthine-guanine phosphoribosyltransferase (HGPRT). How and why this enzyme deficiency leads to abnormal brain development is unknown. To investi...
Gespeichert in:
| Veröffentlicht in: | Neuroscience letters 2001-11, Vol.314 (1), p.61-64 |
|---|---|
| 1. Verfasser: | |
| Format: | Artikel |
| Sprache: | eng |
| Schlagworte: | |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| container_end_page | 64 |
|---|---|
| container_issue | 1 |
| container_start_page | 61 |
| container_title | Neuroscience letters |
| container_volume | 314 |
| creator | Connolly, Gerald P. |
| description | Lesch Nyhan syndrome (LNS) manifests in bizarre and horrific neurological symptoms, the primary cause being a deficiency of the purine salvage enzyme, hypoxanthine-guanine phosphoribosyltransferase (HGPRT). How and why this enzyme deficiency leads to abnormal brain development is unknown. To investigate this phenomenon the present study was designed to examine if the growth of two HGPRT-deficient neuroblastomas, mouse N2aTG and rat B103-4C was different with respect to their corresponding control cell lines, N2a and B103. Data is provided showing that compared to control cell lines, HGPRT-deficient cells proliferated less and exhibited greater morphological complexity. If these abnormalities occur during neurogenesis of human HGPRT-deficient brain neurones, they could profoundly influence central nervous system development and thus, may form the aetiological basis for the symptoms of LNS. |
| doi_str_mv | 10.1016/S0304-3940(01)02290-X |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_72262970</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><els_id>S030439400102290X</els_id><sourcerecordid>72262970</sourcerecordid><originalsourceid>FETCH-LOGICAL-c391t-1cdb1758e9956929d78cf12d615b16dd1dd887481eb8a363de7aaa36308f8e503</originalsourceid><addsrcrecordid>eNqFkcuO1DAQRSMEYpqBTwB5A4JFwJWX49UIjYBBasECkGZnOXalY5TEjcsB8lt8Ie6HmCULqyzdU37UybKnwF8Dh-bNF17yKi9lxV9yeMWLQvL89l62gVYUuZCiuJ9t_iEX2SOi75zzGurqYXYB0MgWKrHJ_tyse_9bz3FwM-a7Rc-psv3gKa3gOk_rGIOeqcegCXOLvTMOZ7OyffB2MUhMdxgSEtmMS3ARmV_iLvhfcWC-Z4nCc-a7UVP0k2YLoWXRsymFI4sDnvLR75zRI7OOfLAY2BbJDOzTOuiZ0Trb4Cd8nD3o9Uj45Fwvs2_v3329vsm3nz98vH67zU0pIeZgbAeiblHKupGFtKI1PRS2gbqDxlqwtm1F1QJ2rS6b0qLQ-rDhbd9izcvL7MXp3PTRHwtSVJMjg-OoZ_QLKVEUTSHFAaxPoAmeKGCv9sFNOqwKuDrIUkdZ6mBCcVBHWeo29T07X7B0E9q7rrOdBDw_A5rSWPo0ZOPojqsAhGzqxF2dOEzj-OkwKDo6QusCmqisd_95yl-ZK7fi</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>72262970</pqid></control><display><type>article</type><title>Hypoxanthine-guanine phosphoribosyltransferase-deficiency produces aberrant neurite outgrowth of rodent neuroblastoma used to model the neurological disorder Lesch Nyhan syndrome</title><source>MEDLINE</source><source>Elsevier ScienceDirect Journals</source><creator>Connolly, Gerald P.</creator><creatorcontrib>Connolly, Gerald P.</creatorcontrib><description>Lesch Nyhan syndrome (LNS) manifests in bizarre and horrific neurological symptoms, the primary cause being a deficiency of the purine salvage enzyme, hypoxanthine-guanine phosphoribosyltransferase (HGPRT). How and why this enzyme deficiency leads to abnormal brain development is unknown. To investigate this phenomenon the present study was designed to examine if the growth of two HGPRT-deficient neuroblastomas, mouse N2aTG and rat B103-4C was different with respect to their corresponding control cell lines, N2a and B103. Data is provided showing that compared to control cell lines, HGPRT-deficient cells proliferated less and exhibited greater morphological complexity. If these abnormalities occur during neurogenesis of human HGPRT-deficient brain neurones, they could profoundly influence central nervous system development and thus, may form the aetiological basis for the symptoms of LNS.</description><identifier>ISSN: 0304-3940</identifier><identifier>EISSN: 1872-7972</identifier><identifier>DOI: 10.1016/S0304-3940(01)02290-X</identifier><identifier>PMID: 11698147</identifier><identifier>CODEN: NELED5</identifier><language>eng</language><publisher>Shannon: Elsevier Ireland Ltd</publisher><subject>Animals ; Biological and medical sciences ; Brain - abnormalities ; Brain - enzymology ; Brain - physiopathology ; Cell Differentiation - genetics ; Cell Division - genetics ; Cell Size - genetics ; Disease Models, Animal ; Errors of metabolism ; Gene Expression Regulation, Developmental - physiology ; Hypoxanthine Phosphoribosyltransferase - deficiency ; Hypoxanthine Phosphoribosyltransferase - genetics ; Hypoxanthine-guanine phosphoribosyltransferase ; Lesch Nyhan syndrome ; Lesch-Nyhan Syndrome - enzymology ; Lesch-Nyhan Syndrome - pathology ; Lesch-Nyhan Syndrome - physiopathology ; Medical sciences ; Metabolic diseases ; Mice ; Morphology ; Neurite outgrowth ; Neurites - enzymology ; Neurites - pathology ; Neuroblastoma ; Proliferation ; Proteins and glycoproteins ; Purine ; Purines - metabolism ; Rats ; Rodent ; Tumor Cells, Cultured - enzymology ; Tumor Cells, Cultured - pathology</subject><ispartof>Neuroscience letters, 2001-11, Vol.314 (1), p.61-64</ispartof><rights>2001 Elsevier Science Ireland Ltd</rights><rights>2002 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c391t-1cdb1758e9956929d78cf12d615b16dd1dd887481eb8a363de7aaa36308f8e503</citedby><cites>FETCH-LOGICAL-c391t-1cdb1758e9956929d78cf12d615b16dd1dd887481eb8a363de7aaa36308f8e503</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/S0304-3940(01)02290-X$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,776,780,3536,27903,27904,45974</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=14117965$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/11698147$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Connolly, Gerald P.</creatorcontrib><title>Hypoxanthine-guanine phosphoribosyltransferase-deficiency produces aberrant neurite outgrowth of rodent neuroblastoma used to model the neurological disorder Lesch Nyhan syndrome</title><title>Neuroscience letters</title><addtitle>Neurosci Lett</addtitle><description>Lesch Nyhan syndrome (LNS) manifests in bizarre and horrific neurological symptoms, the primary cause being a deficiency of the purine salvage enzyme, hypoxanthine-guanine phosphoribosyltransferase (HGPRT). How and why this enzyme deficiency leads to abnormal brain development is unknown. To investigate this phenomenon the present study was designed to examine if the growth of two HGPRT-deficient neuroblastomas, mouse N2aTG and rat B103-4C was different with respect to their corresponding control cell lines, N2a and B103. Data is provided showing that compared to control cell lines, HGPRT-deficient cells proliferated less and exhibited greater morphological complexity. If these abnormalities occur during neurogenesis of human HGPRT-deficient brain neurones, they could profoundly influence central nervous system development and thus, may form the aetiological basis for the symptoms of LNS.</description><subject>Animals</subject><subject>Biological and medical sciences</subject><subject>Brain - abnormalities</subject><subject>Brain - enzymology</subject><subject>Brain - physiopathology</subject><subject>Cell Differentiation - genetics</subject><subject>Cell Division - genetics</subject><subject>Cell Size - genetics</subject><subject>Disease Models, Animal</subject><subject>Errors of metabolism</subject><subject>Gene Expression Regulation, Developmental - physiology</subject><subject>Hypoxanthine Phosphoribosyltransferase - deficiency</subject><subject>Hypoxanthine Phosphoribosyltransferase - genetics</subject><subject>Hypoxanthine-guanine phosphoribosyltransferase</subject><subject>Lesch Nyhan syndrome</subject><subject>Lesch-Nyhan Syndrome - enzymology</subject><subject>Lesch-Nyhan Syndrome - pathology</subject><subject>Lesch-Nyhan Syndrome - physiopathology</subject><subject>Medical sciences</subject><subject>Metabolic diseases</subject><subject>Mice</subject><subject>Morphology</subject><subject>Neurite outgrowth</subject><subject>Neurites - enzymology</subject><subject>Neurites - pathology</subject><subject>Neuroblastoma</subject><subject>Proliferation</subject><subject>Proteins and glycoproteins</subject><subject>Purine</subject><subject>Purines - metabolism</subject><subject>Rats</subject><subject>Rodent</subject><subject>Tumor Cells, Cultured - enzymology</subject><subject>Tumor Cells, Cultured - pathology</subject><issn>0304-3940</issn><issn>1872-7972</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2001</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkcuO1DAQRSMEYpqBTwB5A4JFwJWX49UIjYBBasECkGZnOXalY5TEjcsB8lt8Ie6HmCULqyzdU37UybKnwF8Dh-bNF17yKi9lxV9yeMWLQvL89l62gVYUuZCiuJ9t_iEX2SOi75zzGurqYXYB0MgWKrHJ_tyse_9bz3FwM-a7Rc-psv3gKa3gOk_rGIOeqcegCXOLvTMOZ7OyffB2MUhMdxgSEtmMS3ARmV_iLvhfcWC-Z4nCc-a7UVP0k2YLoWXRsymFI4sDnvLR75zRI7OOfLAY2BbJDOzTOuiZ0Trb4Cd8nD3o9Uj45Fwvs2_v3329vsm3nz98vH67zU0pIeZgbAeiblHKupGFtKI1PRS2gbqDxlqwtm1F1QJ2rS6b0qLQ-rDhbd9izcvL7MXp3PTRHwtSVJMjg-OoZ_QLKVEUTSHFAaxPoAmeKGCv9sFNOqwKuDrIUkdZ6mBCcVBHWeo29T07X7B0E9q7rrOdBDw_A5rSWPo0ZOPojqsAhGzqxF2dOEzj-OkwKDo6QusCmqisd_95yl-ZK7fi</recordid><startdate>20011113</startdate><enddate>20011113</enddate><creator>Connolly, Gerald P.</creator><general>Elsevier Ireland Ltd</general><general>Elsevier</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20011113</creationdate><title>Hypoxanthine-guanine phosphoribosyltransferase-deficiency produces aberrant neurite outgrowth of rodent neuroblastoma used to model the neurological disorder Lesch Nyhan syndrome</title><author>Connolly, Gerald P.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c391t-1cdb1758e9956929d78cf12d615b16dd1dd887481eb8a363de7aaa36308f8e503</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2001</creationdate><topic>Animals</topic><topic>Biological and medical sciences</topic><topic>Brain - abnormalities</topic><topic>Brain - enzymology</topic><topic>Brain - physiopathology</topic><topic>Cell Differentiation - genetics</topic><topic>Cell Division - genetics</topic><topic>Cell Size - genetics</topic><topic>Disease Models, Animal</topic><topic>Errors of metabolism</topic><topic>Gene Expression Regulation, Developmental - physiology</topic><topic>Hypoxanthine Phosphoribosyltransferase - deficiency</topic><topic>Hypoxanthine Phosphoribosyltransferase - genetics</topic><topic>Hypoxanthine-guanine phosphoribosyltransferase</topic><topic>Lesch Nyhan syndrome</topic><topic>Lesch-Nyhan Syndrome - enzymology</topic><topic>Lesch-Nyhan Syndrome - pathology</topic><topic>Lesch-Nyhan Syndrome - physiopathology</topic><topic>Medical sciences</topic><topic>Metabolic diseases</topic><topic>Mice</topic><topic>Morphology</topic><topic>Neurite outgrowth</topic><topic>Neurites - enzymology</topic><topic>Neurites - pathology</topic><topic>Neuroblastoma</topic><topic>Proliferation</topic><topic>Proteins and glycoproteins</topic><topic>Purine</topic><topic>Purines - metabolism</topic><topic>Rats</topic><topic>Rodent</topic><topic>Tumor Cells, Cultured - enzymology</topic><topic>Tumor Cells, Cultured - pathology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Connolly, Gerald P.</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Neuroscience letters</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Connolly, Gerald P.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Hypoxanthine-guanine phosphoribosyltransferase-deficiency produces aberrant neurite outgrowth of rodent neuroblastoma used to model the neurological disorder Lesch Nyhan syndrome</atitle><jtitle>Neuroscience letters</jtitle><addtitle>Neurosci Lett</addtitle><date>2001-11-13</date><risdate>2001</risdate><volume>314</volume><issue>1</issue><spage>61</spage><epage>64</epage><pages>61-64</pages><issn>0304-3940</issn><eissn>1872-7972</eissn><coden>NELED5</coden><abstract>Lesch Nyhan syndrome (LNS) manifests in bizarre and horrific neurological symptoms, the primary cause being a deficiency of the purine salvage enzyme, hypoxanthine-guanine phosphoribosyltransferase (HGPRT). How and why this enzyme deficiency leads to abnormal brain development is unknown. To investigate this phenomenon the present study was designed to examine if the growth of two HGPRT-deficient neuroblastomas, mouse N2aTG and rat B103-4C was different with respect to their corresponding control cell lines, N2a and B103. Data is provided showing that compared to control cell lines, HGPRT-deficient cells proliferated less and exhibited greater morphological complexity. If these abnormalities occur during neurogenesis of human HGPRT-deficient brain neurones, they could profoundly influence central nervous system development and thus, may form the aetiological basis for the symptoms of LNS.</abstract><cop>Shannon</cop><pub>Elsevier Ireland Ltd</pub><pmid>11698147</pmid><doi>10.1016/S0304-3940(01)02290-X</doi><tpages>4</tpages></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0304-3940 |
| ispartof | Neuroscience letters, 2001-11, Vol.314 (1), p.61-64 |
| issn | 0304-3940 1872-7972 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_72262970 |
| source | MEDLINE; Elsevier ScienceDirect Journals |
| subjects | Animals Biological and medical sciences Brain - abnormalities Brain - enzymology Brain - physiopathology Cell Differentiation - genetics Cell Division - genetics Cell Size - genetics Disease Models, Animal Errors of metabolism Gene Expression Regulation, Developmental - physiology Hypoxanthine Phosphoribosyltransferase - deficiency Hypoxanthine Phosphoribosyltransferase - genetics Hypoxanthine-guanine phosphoribosyltransferase Lesch Nyhan syndrome Lesch-Nyhan Syndrome - enzymology Lesch-Nyhan Syndrome - pathology Lesch-Nyhan Syndrome - physiopathology Medical sciences Metabolic diseases Mice Morphology Neurite outgrowth Neurites - enzymology Neurites - pathology Neuroblastoma Proliferation Proteins and glycoproteins Purine Purines - metabolism Rats Rodent Tumor Cells, Cultured - enzymology Tumor Cells, Cultured - pathology |
| title | Hypoxanthine-guanine phosphoribosyltransferase-deficiency produces aberrant neurite outgrowth of rodent neuroblastoma used to model the neurological disorder Lesch Nyhan syndrome |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-21T12%3A23%3A51IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_cross&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Hypoxanthine-guanine%20phosphoribosyltransferase-deficiency%20produces%20aberrant%20neurite%20outgrowth%20of%20rodent%20neuroblastoma%20used%20to%20model%20the%20neurological%20disorder%20Lesch%20Nyhan%20syndrome&rft.jtitle=Neuroscience%20letters&rft.au=Connolly,%20Gerald%20P.&rft.date=2001-11-13&rft.volume=314&rft.issue=1&rft.spage=61&rft.epage=64&rft.pages=61-64&rft.issn=0304-3940&rft.eissn=1872-7972&rft.coden=NELED5&rft_id=info:doi/10.1016/S0304-3940(01)02290-X&rft_dat=%3Cproquest_cross%3E72262970%3C/proquest_cross%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=72262970&rft_id=info:pmid/11698147&rft_els_id=S030439400102290X&rfr_iscdi=true |