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| Title : Moving towards effective therapeutic strategies for Neuronal Ceroid Lipofuscinosis - Geraets_2016_Orphanet.J.Rare.Dis_11_40 |
| Author(s) : Geraets RD , Koh S , Hastings ML , Kielian T , Pearce DA , Weimer JM |
| Ref : Orphanet J Rare Dis , 11 :40 , 2016 |
| Abstract : Geraets_2016_Orphanet.J.Rare.Dis_11_40 |
| ESTHER : Geraets_2016_Orphanet.J.Rare.Dis_11_40 |
| PubMedSearch : Geraets_2016_Orphanet.J.Rare.Dis_11_40 |
| PubMedID: 27083890 |
| Title : Tissue-specific variation in nonsense mutant transcript level and drug-induced read-through efficiency in the Cln1(R151X) mouse model of INCL - Thada_2016_J.Cell.Mol.Med_20_381 |
| Author(s) : Thada V , Miller JN , Kovacs AD , Pearce DA |
| Ref : J Cell Mol Med , 20 :381 , 2016 |
| Abstract : Thada_2016_J.Cell.Mol.Med_20_381 |
| ESTHER : Thada_2016_J.Cell.Mol.Med_20_381 |
| PubMedSearch : Thada_2016_J.Cell.Mol.Med_20_381 |
| PubMedID: 26648046 |
| Gene_locus related to this paper: mouse-ppt |
| Title : The novel Cln1(R151X) mouse model of infantile neuronal ceroid lipofuscinosis (INCL) for testing nonsense suppression therapy - Miller_2015_Hum.Mol.Genet_24_185 |
| Author(s) : Miller JN , Kovacs AD , Pearce DA |
| Ref : Hum Mol Genet , 24 :185 , 2015 |
| Abstract : Miller_2015_Hum.Mol.Genet_24_185 |
| ESTHER : Miller_2015_Hum.Mol.Genet_24_185 |
| PubMedSearch : Miller_2015_Hum.Mol.Genet_24_185 |
| PubMedID: 25205113 |
| Title : Treatment of the Ppt1(-\/-) mouse model of infantile neuronal ceroid lipofuscinosis with the N-methyl-D-aspartate (NMDA) receptor antagonist memantine - Finn_2013_J.Child.Neurol_28_1159 |
| Author(s) : Finn R , Kovacs AD , Pearce DA |
| Ref : Journal of Child Neurology , 28 :1159 , 2013 |
| Abstract : Finn_2013_J.Child.Neurol_28_1159 |
| ESTHER : Finn_2013_J.Child.Neurol_28_1159 |
| PubMedSearch : Finn_2013_J.Child.Neurol_28_1159 |
| PubMedID: 24014511 |
| Gene_locus related to this paper: mouse-ppt |
| Title : The role of nonsense-mediated decay in neuronal ceroid lipofuscinosis - Miller_2013_Hum.Mol.Genet_22_2723 |
| Author(s) : Miller JN , Chan CH , Pearce DA |
| Ref : Hum Mol Genet , 22 :2723 , 2013 |
| Abstract : Miller_2013_Hum.Mol.Genet_22_2723 |
| ESTHER : Miller_2013_Hum.Mol.Genet_22_2723 |
| PubMedSearch : Miller_2013_Hum.Mol.Genet_22_2723 |
| PubMedID: 23539563 |
| Title : A novel c.776_777insA mutation in CLN1 leads to infantile neuronal ceroid lipofuscinosis - Miller_2013_J.Child.Neurol_28_1106 |
| Author(s) : Miller JN , Pearce DA |
| Ref : Journal of Child Neurology , 28 :1106 , 2013 |
| Abstract : Miller_2013_J.Child.Neurol_28_1106 |
| ESTHER : Miller_2013_J.Child.Neurol_28_1106 |
| PubMedSearch : Miller_2013_J.Child.Neurol_28_1106 |
| PubMedID: 23857568 |
| Gene_locus related to this paper: human-PPT1 |
| Title : Altered glutamate receptor function in the cerebellum of the Ppt1-\/- mouse, a murine model of infantile neuronal ceroid lipofuscinosis - Finn_2012_J.Neurosci.Res_90_367 |
| Author(s) : Finn R , Kovacs AD , Pearce DA |
| Ref : Journal of Neuroscience Research , 90 :367 , 2012 |
| Abstract : Finn_2012_J.Neurosci.Res_90_367 |
| ESTHER : Finn_2012_J.Neurosci.Res_90_367 |
| PubMedSearch : Finn_2012_J.Neurosci.Res_90_367 |
| PubMedID: 21971706 |
| Gene_locus related to this paper: mouse-ppt |
| Title : Interactions of the proteins of neuronal ceroid lipofuscinosis: clues to function - Getty_2011_Cell.Mol.Life.Sci_68_453 |
| Author(s) : Getty AL , Pearce DA |
| Ref : Cell Mol Life Sciences , 68 :453 , 2011 |
| Abstract : Getty_2011_Cell.Mol.Life.Sci_68_453 |
| ESTHER : Getty_2011_Cell.Mol.Life.Sci_68_453 |
| PubMedSearch : Getty_2011_Cell.Mol.Life.Sci_68_453 |
| PubMedID: 20680390 |
| Gene_locus related to this paper: human-PPT1 |
| Title : Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease - Kielar_2009_Hum.Mol.Genet_18_4066 |
| Author(s) : Kielar C , Wishart TM , Palmer A , Dihanich S , Wong AM , Macauley SL , Chan CH , Sands MS , Pearce DA , Cooper JD , Gillingwater TH |
| Ref : Hum Mol Genet , 18 :4066 , 2009 |
| Abstract : Kielar_2009_Hum.Mol.Genet_18_4066 |
| ESTHER : Kielar_2009_Hum.Mol.Genet_18_4066 |
| PubMedSearch : Kielar_2009_Hum.Mol.Genet_18_4066 |
| PubMedID: 19640925 |
| Gene_locus related to this paper: mouse-ppt |
| Title : Characterizing pathogenic processes in Batten disease: use of small eukaryotic model systems - Phillips_2006_Biochim.Biophys.Acta_1762_906 |
| Author(s) : Phillips SN , Muzaffar N , Codlin S , Korey CA , Taschner PE , de Voer G , Mole SE , Pearce DA |
| Ref : Biochimica & Biophysica Acta , 1762 :906 , 2006 |
| Abstract : Phillips_2006_Biochim.Biophys.Acta_1762_906 |
| ESTHER : Phillips_2006_Biochim.Biophys.Acta_1762_906 |
| PubMedSearch : Phillips_2006_Biochim.Biophys.Acta_1762_906 |
| PubMedID: 17049819 |
| Gene_locus related to this paper: caeel-F44C4.5 , drome-CG12108 |
| Title : Early changes in gene expression in two models of Batten disease - Elshatory_2003_FEBS.Lett_538_207 |
| Author(s) : Elshatory Y , Brooks AI , Chattopadhyay S , Curran TM , Gupta P , Ramalingam V , Hofmann SL , Pearce DA |
| Ref : FEBS Letters , 538 :207 , 2003 |
| Abstract : Elshatory_2003_FEBS.Lett_538_207 |
| ESTHER : Elshatory_2003_FEBS.Lett_538_207 |
| PubMedSearch : Elshatory_2003_FEBS.Lett_538_207 |
| PubMedID: 12633880 |
| Gene_locus related to this paper: mouse-ppt |