title: Doppel expression is regulated by the Brn-3a and Brn-3b transcription factors.
abstract: Doppel and the prion protein (PrP) are two related proteins involved in different aspects of neuronal degeneration. While a structural modification of PrP is necessary and sufficient for its toxic effect, the neurotoxicity of Doppel in the Purkinje cells of the cerebellum relies solely on its overexpression. Understanding the Doppel-related neurotoxicity thus involves the analysis of its developmental and transcriptional regulation. Here we report for the first time that Doppel is expressed in the embryonic neurons of mice dorsal root ganglia and spinal cord and that the closely related Brn-3a and Brn-3b transcription factors are involved in its transcriptional regulation.
authors.affiliation: MMBU, Institute of Child Health, University College London 30 Guilford Street, London WC1N 1EH, UK. m.calissano@ich.ucl.ac.uk
authors.forename: M
authors.initials: M
authors.lastname: Calissano
authors.forename: E
authors.initials: E
authors.lastname: Ensor
authors.forename: D R
authors.initials: DR
authors.lastname: Brown
authors.forename: D S
authors.initials: DS
authors.lastname: Latchman
mesh_terms: D000818:Animals; D015153:Blotting, Western; D001932:Brain Neoplasms / Q000378:metabolism; D045744:Cell Line, Tumor; D002478:Cells, Cultured; D004268:DNA-Binding Proteins / Q000235:genetics*; D058851:GPI-Linked Proteins; D005727:Ganglia, Spinal / Q000196:embryology / Q000378:metabolism; D005786:Gene Expression Regulation / Q000502:physiology; D006801:Humans; D017403:In Situ Hybridization; D051379:Mice; D018345:Mice, Knockout; D009447:Neuroblastoma / Q000378:metabolism; D011328:Prions / Q000096:biosynthesis* / Q000235:genetics; D012333:RNA, Messenger / Q000096:biosynthesis / Q000235:genetics; D020133:Reverse Transcriptase Polymerase Chain Reaction; D013116:Spinal Cord / Q000196:embryology / Q000378:metabolism; D050818:Transcription Factor Brn-3; D050819:Transcription Factor Brn-3A; D050820:Transcription Factor Brn-3B; D014157:Transcription Factors / Q000235:genetics*; D014162:Transfection
chemical_list: D004268:DNA-Binding Proteins; D058851:GPI-Linked Proteins; C494188:POU4F1 protein, human; C494190:POU4F2 protein, human; C501125:PRND protein, human; C494189:Pou4f1 protein, mouse; D011328:Prions; C122036:Prnd protein, mouse; D012333:RNA, Messenger; D050818:Transcription Factor Brn-3; D050819:Transcription Factor Brn-3A; D050820:Transcription Factor Brn-3B; D014157:Transcription Factors