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Search results 101 to 165 out of 165 for Ncl

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Category: Publication
Type Details Score
Publication      
First Author: Mouse Genome Informatics
Year: 2010
Journal: Database Release
Title: Protein Ontology Association Load.
Publication        
First Author: Mouse Genome Informatics Scientific Curators
Year: 2005
Title: Obtaining and loading genome assembly coordinates from NCBI annotations
Publication      
First Author: Mouse Genome Informatics Scientific Curators
Year: 2009
Journal: Database Download
Title: Mouse Microarray Data Integration in Mouse Genome Informatics, the Affymetrix GeneChip Mouse Genome 430 2.0 Array Platform
Publication
First Author: Cárcel-Trullols J
Year: 2015
Journal: Biochim Biophys Acta
Title: Cell biology of the NCL proteins: What they do and don't do.
Volume: 1852
Issue: 10 Pt B
Pages: 2242-55
Publication
First Author: Jalanko A
Year: 2006
Journal: Biochim Biophys Acta
Title: From genes to systems: new global strategies for the characterization of NCL biology.
Volume: 1762
Issue: 10
Pages: 934-44
Publication  
First Author: von Schantz C
Year: 2008
Journal: BMC Genomics
Title: Brain gene expression profiles of Cln1 and Cln5 deficient mice unravels common molecular pathways underlying neuronal degeneration in NCL diseases.
Volume: 9
Pages: 146
Publication
First Author: Lührig S
Year: 2014
Journal: Stem Cells Dev
Title: Lrrc34, a novel nucleolar protein, interacts with npm1 and ncl and has an impact on pluripotent stem cells.
Volume: 23
Issue: 23
Pages: 2862-74
Publication
First Author: Zhou X
Year: 2015
Journal: J Cell Biol
Title: Prosaposin facilitates sortilin-independent lysosomal trafficking of progranulin.
Volume: 210
Issue: 6
Pages: 991-1002
Publication  
First Author: Zhou X
Year: 2017
Journal: Nat Commun
Title: Impaired prosaposin lysosomal trafficking in frontotemporal lobar degeneration due to progranulin mutations.
Volume: 8
Pages: 15277
Publication
First Author: Messer A
Year: 1999
Journal: Mol Genet Metab
Title: An early-onset congenic strain of the motor neuron degeneration (mnd) mouse.
Volume: 66
Issue: 4
Pages: 393-7
Publication
First Author: Seigel GM
Year: 2005
Journal: Eye (Lond)
Title: Progression of early postnatal retinal pathology in a mouse model of neuronal ceroid lipofuscinosis.
Volume: 19
Issue: 12
Pages: 1306-12
Publication
First Author: Elshatory Y
Year: 2003
Journal: FEBS Lett
Title: Early changes in gene expression in two models of Batten disease.
Volume: 538
Issue: 1-3
Pages: 207-12
Publication
First Author: Pontikis CC
Year: 2004
Journal: Brain Res
Title: Late onset neurodegeneration in the Cln3-/- mouse model of juvenile neuronal ceroid lipofuscinosis is preceded by low level glial activation.
Volume: 1023
Issue: 2
Pages: 231-42
Publication
First Author: Wheeler RB
Year: 2002
Journal: Am J Hum Genet
Title: The gene mutated in variant late-infantile neuronal ceroid lipofuscinosis (CLN6) and in nclf mutant mice encodes a novel predicted transmembrane protein.
Volume: 70
Issue: 2
Pages: 537-42
Publication
First Author: Holmberg V
Year: 2004
Journal: Neurobiol Dis
Title: The mouse ortholog of the neuronal ceroid lipofuscinosis CLN5 gene encodes a soluble lysosomal glycoprotein expressed in the developing brain.
Volume: 16
Issue: 1
Pages: 29-40
Publication
First Author: Chang MC
Year: 2017
Journal: J Exp Med
Title: Progranulin deficiency causes impairment of autophagy and TDP-43 accumulation.
Volume: 214
Issue: 9
Pages: 2611-2628
Publication
First Author: Wang L
Year: 2021
Journal: Nat Commun
Title: A novel lncRNA Discn fine-tunes replication protein A (RPA) availability to promote genomic stability.
Volume: 12
Issue: 1
Pages: 5572
Publication
First Author: Wang Y
Year: 2021
Journal: Sci Adv
Title: CLN7 is an organellar chloride channel regulating lysosomal function.
Volume: 7
Issue: 51
Pages: eabj9608
Publication  
First Author: Gueiderikh A
Year: 2021
Journal: Sci Adv
Title: Fanconi anemia A protein participates in nucleolar homeostasis maintenance and ribosome biogenesis.
Volume: 7
Issue: 1
Publication  
First Author: Lyly A
Year: 2009
Journal: BMC Cell Biol
Title: Novel interactions of CLN5 support molecular networking between Neuronal Ceroid Lipofuscinosis proteins.
Volume: 10
Pages: 83
Publication
First Author: Staropoli JF
Year: 2012
Journal: Am J Hum Genet
Title: A homozygous mutation in KCTD7 links neuronal ceroid lipofuscinosis to the ubiquitin-proteasome system.
Volume: 91
Issue: 1
Pages: 202-8
Publication
First Author: Poët M
Year: 2006
Journal: Proc Natl Acad Sci U S A
Title: Lysosomal storage disease upon disruption of the neuronal chloride transport protein ClC-6.
Volume: 103
Issue: 37
Pages: 13854-9
Publication
First Author: Ranta S
Year: 1999
Journal: Nat Genet
Title: The neuronal ceroid lipofuscinoses in human EPMR and mnd mutant mice are associated with mutations in CLN8.
Volume: 23
Issue: 2
Pages: 233-6
Publication
First Author: Geraets RD
Year: 2017
Journal: PLoS One
Title: A tailored mouse model of CLN2 disease: A nonsense mutant for testing personalized therapies.
Volume: 12
Issue: 5
Pages: e0176526
Publication
First Author: Faust JR
Year: 1994
Journal: J Biol Chem
Title: Two related proteolipids and dolichol-linked oligosaccharides accumulate in motor neuron degeneration mice (mnd/mnd), a model for neuronal ceroid lipofuscinosis.
Volume: 269
Issue: 13
Pages: 10150-5
Publication
First Author: Cho SK
Year: 2005
Journal: Glycobiology
Title: Characterization of lipid-linked oligosaccharide accumulation in mouse models of Batten disease.
Volume: 15
Issue: 6
Pages: 637-48
Publication
First Author: Pardo CA
Year: 1994
Journal: Am J Pathol
Title: Accumulation of the adenosine triphosphate synthase subunit C in the mnd mutant mouse. A model for neuronal ceroid lipofuscinosis.
Volume: 144
Issue: 4
Pages: 829-35
Publication
First Author: Wendt KD
Year: 2005
Journal: Behav Brain Res
Title: Behavioral assessment in mouse models of neuronal ceroid lipofuscinosis using a light-cued T-maze.
Volume: 161
Issue: 2
Pages: 175-82
Publication
First Author: Guarneri R
Year: 2004
Journal: Brain Res
Title: Retinal oxidation, apoptosis and age- and sex-differences in the mnd mutant mouse, a model of neuronal ceroid lipofuscinosis.
Volume: 1014
Issue: 1-2
Pages: 209-20
Publication
First Author: Bermingham NA
Year: 1996
Journal: Genomics
Title: The mouse lysosomal membrane protein 1 gene as a candidate for the motorneuron degeneration (mnd) locus.
Volume: 32
Issue: 2
Pages: 266-71
Publication
First Author: Griffin JL
Year: 2002
Journal: Physiol Genomics
Title: Vitamin E deficiency and metabolic deficits in neuronal ceroid lipofuscinosis described by bioinformatics.
Volume: 11
Issue: 3
Pages: 195-203
Publication
First Author: Hersrud SL
Year: 2016
Journal: Biochim Biophys Acta
Title: Antigen presenting cell abnormalities in the Cln3(-/-) mouse model of juvenile neuronal ceroid lipofuscinosis.
Volume: 1862
Issue: 7
Pages: 1324-36
Publication
First Author: Pears MR
Year: 2005
Journal: J Biol Chem
Title: High resolution 1H NMR-based metabolomics indicates a neurotransmitter cycling deficit in cerebral tissue from a mouse model of Batten disease.
Volume: 280
Issue: 52
Pages: 42508-14
Publication  
First Author: Ketscher A
Year: 2016
Journal: Biochimie
Title: Neuroectoderm-specific deletion of cathepsin D in mice models human inherited neuronal ceroid lipofuscinosis type 10.
Volume: 122
Pages: 219-26
Publication
First Author: Kielar C
Year: 2009
Journal: Hum Mol Genet
Title: Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease.
Volume: 18
Issue: 21
Pages: 4066-80
Publication
First Author: Kleine Holthaus SM
Year: 2018
Journal: Mol Ther
Title: Prevention of Photoreceptor Cell Loss in a Cln6nclf Mouse Model of Batten Disease Requires CLN6 Gene Transfer to Bipolar Cells.
Volume: 26
Issue: 5
Pages: 1343-1353
Publication
First Author: Gao H
Year: 2002
Journal: Am J Hum Genet
Title: Mutations in a novel CLN6-encoded transmembrane protein cause variant neuronal ceroid lipofuscinosis in man and mouse.
Volume: 70
Issue: 2
Pages: 324-35
Publication
First Author: Morgan JP
Year: 2013
Journal: PLoS One
Title: A murine model of variant late infantile ceroid lipofuscinosis recapitulates behavioral and pathological phenotypes of human disease.
Volume: 8
Issue: 11
Pages: e78694
Publication
First Author: Gorenberg EL
Year: 2022
Journal: PLoS Biol
Title: Identification of substrates of palmitoyl protein thioesterase 1 highlights roles of depalmitoylation in disulfide bond formation and synaptic function.
Volume: 20
Issue: 3
Pages: e3001590
Publication
First Author: Finn R
Year: 2012
Journal: J Neurosci Res
Title: Altered glutamate receptor function in the cerebellum of the Ppt1-/- mouse, a murine model of infantile neuronal ceroid lipofuscinosis.
Volume: 90
Issue: 2
Pages: 367-75
Publication
First Author: Hsu TI
Year: 2015
Journal: Oncogene
Title: MMP7-mediated cleavage of nucleolin at Asp255 induces MMP9 expression to promote tumor malignancy.
Volume: 34
Issue: 7
Pages: 826-37
Publication
First Author: Tsoi H
Year: 2012
Journal: Proc Natl Acad Sci U S A
Title: CAG expansion induces nucleolar stress in polyglutamine diseases.
Volume: 109
Issue: 33
Pages: 13428-33
Publication
First Author: Savchenko E
Year: 2017
Journal: Dis Model Mech
Title: Loss of Cln5 causes altered neurogenesis in a mouse model of a childhood neurodegenerative disorder.
Volume: 10
Issue: 9
Pages: 1089-1100
Publication
First Author: Blom T
Year: 2013
Journal: Dis Model Mech
Title: Exacerbated neuronal ceroid lipofuscinosis phenotype in Cln1/5 double-knockout mice.
Volume: 6
Issue: 2
Pages: 342-57
Publication  
First Author: Doccini S
Year: 2022
Journal: Cells
Title: Lysosomal Proteomics Links Disturbances in Lipid Homeostasis and Sphingolipid Metabolism to CLN5 Disease.
Volume: 11
Issue: 11
Publication  
First Author: Hafler BP
Year: 2014
Journal: Brain Res
Title: Progressive retinal degeneration and accumulation of autofluorescent lipopigments in Progranulin deficient mice.
Volume: 1588
Pages: 168-74
Publication  
First Author: Ward ME
Year: 2017
Journal: Sci Transl Med
Title: Individuals with progranulin haploinsufficiency exhibit features of neuronal ceroid lipofuscinosis.
Volume: 9
Issue: 385
Publication
First Author: Schultheis PJ
Year: 2013
Journal: Hum Mol Genet
Title: Atp13a2-deficient mice exhibit neuronal ceroid lipofuscinosis, limited α-synuclein accumulation and age-dependent sensorimotor deficits.
Volume: 22
Issue: 10
Pages: 2067-82
Publication
First Author: Miller JN
Year: 2015
Journal: Hum Mol Genet
Title: The novel Cln1(R151X) mouse model of infantile neuronal ceroid lipofuscinosis (INCL) for testing nonsense suppression therapy.
Volume: 24
Issue: 1
Pages: 185-96
Publication
First Author: Sharifi A
Year: 2010
Journal: Hum Mol Genet
Title: Expression and lysosomal targeting of CLN7, a major facilitator superfamily transporter associated with variant late-infantile neuronal ceroid lipofuscinosis.
Volume: 19
Issue: 22
Pages: 4497-514
Publication
First Author: Broom MF
Year: 1998
Journal: J Med Genet
Title: Ovine neuronal ceroid lipofuscinosis: a large animal model syntenic with the human neuronal ceroid lipofuscinosis variant CLN6.
Volume: 35
Issue: 9
Pages: 717-21
Publication  
First Author: Lonka L
Year: 2005
Journal: BMC Neurosci
Title: The neuronal ceroid lipofuscinosis Cln8 gene expression is developmentally regulated in mouse brain and up-regulated in the hippocampal kindling model of epilepsy.
Volume: 6
Pages: 27
Publication
First Author: Peddigari S
Year: 2013
Journal: Nucleic Acids Res
Title: hnRNPL and nucleolin bind LINE-1 RNA and function as host factors to modulate retrotransposition.
Volume: 41
Issue: 1
Pages: 575-85
Publication
First Author: Gongol B
Year: 2019
Journal: Proc Natl Acad Sci U S A
Title: Shear stress regulation of miR-93 and miR-484 maturation through nucleolin.
Volume: 116
Issue: 26
Pages: 12974-12979
Publication
First Author: Appu AP
Year: 2019
Journal: J Inherit Metab Dis
Title: Cln3-mutations underlying juvenile neuronal ceroid lipofuscinosis cause significantly reduced levels of Palmitoyl-protein thioesterases-1 (Ppt1)-protein and Ppt1-enzyme activity in the lysosome.
Volume: 42
Issue: 5
Pages: 944-954
Publication
First Author: Ookuma YF
Year: 2013
Journal: Histochem Cell Biol
Title: Multiple functional involvement of thymosin beta-4 in tooth germ development.
Volume: 139
Issue: 2
Pages: 355-70
Publication
First Author: Fabritius AL
Year: 2014
Journal: Exp Mol Pathol
Title: Neuronal ceroid lipofuscinosis genes, CLN2, CLN3 and CLN5 are spatially and temporally co-expressed in a developing mouse brain.
Volume: 97
Issue: 3
Pages: 484-91
Publication
First Author: Bronson RT
Year: 1998
Journal: Am J Med Genet
Title: Neuronal ceroid lipofuscinosis (nclf), a new disorder of the mouse linked to chromosome 9.
Volume: 77
Issue: 4
Pages: 289-97
Publication
First Author: Shacka JJ
Year: 2007
Journal: J Neurosci
Title: Cathepsin D deficiency induces persistent neurodegeneration in the absence of Bax-dependent apoptosis.
Volume: 27
Issue: 8
Pages: 2081-90
Publication
First Author: Mirza M
Year: 2013
Journal: PLoS One
Title: Progressive retinal degeneration and glial activation in the CLN6 (nclf) mouse model of neuronal ceroid lipofuscinosis: a beneficial effect of DHA and curcumin supplementation.
Volume: 8
Issue: 10
Pages: e75963
Publication
First Author: Lange J
Year: 2018
Journal: Acta Neuropathol Commun
Title: Compromised astrocyte function and survival negatively impact neurons in infantile neuronal ceroid lipofuscinosis.
Volume: 6
Issue: 1
Pages: 74
Publication
First Author: Domowicz MS
Year: 2021
Journal: J Neuroinflammation
Title: Brain transcriptome analysis of a CLN2 mouse model as a function of disease progression.
Volume: 18
Issue: 1
Pages: 262
Publication
First Author: Cooper JD
Year: 2006
Journal: Biochim Biophys Acta
Title: Progress towards understanding disease mechanisms in small vertebrate models of neuronal ceroid lipofuscinosis.
Volume: 1762
Issue: 10
Pages: 873-89
Publication
First Author: Arrant AE
Year: 2018
Journal: J Neurosci
Title: Progranulin Gene Therapy Improves Lysosomal Dysfunction and Microglial Pathology Associated with Frontotemporal Dementia and Neuronal Ceroid Lipofuscinosis.
Volume: 38
Issue: 9
Pages: 2341-2358
Publication
First Author: Lu JY
Year: 2015
Journal: Mol Genet Metab
Title: Intrathecal enzyme replacement therapy improves motor function and survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis.
Volume: 116
Issue: 1-2
Pages: 98-105