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Search results 1 to 100 out of 463 for Ppt1

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Hits by Pathway

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Hits by Strain

Type Details Score
Gene
5538 | PPT1
Type: gene
Organism: human
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Gene
448650 | ppt1
Type: gene
Organism: frog, western clawed
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Gene
29411 | Ppt1
Type: gene
Organism: rat
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Gene
475316 | PPT1
Type: gene
Organism: dog, domestic
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Gene
456789 | PPT1
Type: gene
Organism: chimpanzee
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Gene
281421 | PPT1
Type: gene
Organism: cattle
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Gene
419681 | PPT1
Type: gene
Organism: chicken
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Gene
406648 | ppt1
Type: gene
Organism: zebrafish
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Gene
693377 | PPT1
Type: gene
Organism: macaque, rhesus
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Protein Domain
IPR030294 | PPT1
Type: Family
Description: Palmitoyl-protein thioesterase 1 (PPT1) is responsible for the removal of a palmitate group from its substrate proteins, which may include presynaptic proteins like SNAP-25, cysteine string protein (CSP), dynamin, and synaptotagmin []. Mutations in the PPT1 gene cause infantile-onset neuronal ceroid lipofuscinosis (INCL), which is a severe pediatric neurodegenerative disorder []. This entry also includes uncharacterised proteins from fungi and plants.
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Protein Coding Gene
MGI:1298204 | Ppt1
Type: protein_coding_gene
Organism: mouse, laboratory
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Cell Line Derivation
Cell Line Derivation
Derivation Type: Gene trapped
Name: CMHD Gene trapped Library C2 (Tcp) C57BL/6N pPT1
Creator: CMHD
Vector Type: Not Specified
Vector: pPT1
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Cell Line Derivation
Cell Line Derivation
Derivation Type: Gene trapped
Name: CMHD Gene Trap Library GTL_R1_pPT1
Creator: CMHD
Vector Type: Not Specified
Vector: pPT1
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Publication
31262842 | J:301929
First Author: Ou P
Year: 2019
Journal: J Exp Med
Title: Thioesterase PPT1 balances viral resistance and efficient T cell crosspriming in dendritic cells.
Volume: 216
Issue: 9
Pages: 2091-2112
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Publication
35628400 | J:325695
 
First Author: Zhang X
Year: 2022
Journal: Int J Mol Sci
Title: Seizures in PPT1 Knock-In Mice Are Associated with Inflammatory Activation of Microglia.
Volume: 23
Issue: 10
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Publication
24091420 | -
First Author: Aby E
Year: 2013
Journal: Fly (Austin)
Title: Mutations in palmitoyl-protein thioesterase 1 alter exocytosis and endocytosis at synapses in Drosophila larvae.
Volume: 7
Issue: 4
Pages: 267-79
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Publication
23747979 | -
First Author: Hawkins-Salsbury JA
Year: 2013
Journal: Biochim Biophys Acta
Title: Pathogenesis and therapies for infantile neuronal ceroid lipofuscinosis (infantile CLN1 disease).
Volume: 1832
Issue: 11
Pages: 1906-9
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Publication
11717424 | J:72931
First Author: Gupta P
Year: 2001
Journal: Proc Natl Acad Sci U S A
Title: Disruption of PPT1 or PPT2 causes neuronal ceroid lipofuscinosis in knockout mice.
Volume: 98
Issue: 24
Pages: 13566-71
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Publication
17341491 | J:121809
First Author: Zhang Z
Year: 2007
Journal: Hum Mol Genet
Title: Production of lysophosphatidylcholine by cPLA2 in the brain of mice lacking PPT1 is a signal for phagocyte infiltration.
Volume: 16
Issue: 7
Pages: 837-47
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Protein
O88531
Organism: Mus musculus/domesticus
Length: 306  
Fragment?: false
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Protein
Q3U6J9
Organism: Mus musculus/domesticus
Length: 306  
Fragment?: false
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Protein
Q3UB92
Organism: Mus musculus/domesticus
Length: 306  
Fragment?: false
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Protein
B1B0P9
Organism: Mus musculus/domesticus
Length: 197  
Fragment?: false
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Protein
Q3UFT5
Organism: Mus musculus/domesticus
Length: 197  
Fragment?: false
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Protein
B1B0P8
Organism: Mus musculus/domesticus
Length: 179  
Fragment?: false
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Protein
Q3TEL0
Organism: Mus musculus/domesticus
Length: 302  
Fragment?: false
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Protein
Q3TAR8
Organism: Mus musculus/domesticus
Length: 306  
Fragment?: false
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Protein
Q8VBX5
Organism: Mus musculus/domesticus
Length: 307  
Fragment?: false
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Protein
Q3U6W3
Organism: Mus musculus/domesticus
Length: 306  
Fragment?: false
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Publication
18245779 | J:135269
First Author: Lyly A
Year: 2008
Journal: Hum Mol Genet
Title: Deficiency of the INCL protein Ppt1 results in changes in ectopic F1-ATP synthase and altered cholesterol metabolism.
Volume: 17
Issue: 10
Pages: 1406-17
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Publication
17046272 | J:134738
First Author: Kielar C
Year: 2007
Journal: Neurobiol Dis
Title: Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis.
Volume: 25
Issue: 1
Pages: 150-62
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Publication
19733542 | J:154123
First Author: Tamaki SJ
Year: 2009
Journal: Cell Stem Cell
Title: Neuroprotection of host cells by human central nervous system stem cells in a mouse model of infantile neuronal ceroid lipofuscinosis.
Volume: 5
Issue: 3
Pages: 310-9
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Publication
24056696 | J:207427
First Author: Sarkar C
Year: 2013
Journal: Nat Neurosci
Title: Neuroprotection and lifespan extension in Ppt1(-/-) mice by NtBuHA: therapeutic implications for INCL.
Volume: 16
Issue: 11
Pages: 1608-17
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Publication
15649713 | J:95522
First Author: Jalanko A
Year: 2005
Journal: Neurobiol Dis
Title: Mice with Ppt1Deltaex4 mutation replicate the INCL phenotype and show an inflammation-associated loss of interneurons.
Volume: 18
Issue: 1
Pages: 226-41
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Publication
25574475 | J:261869
First Author: Bouchelion A
Year: 2014
Journal: Ann Clin Transl Neurol
Title: Mice homozygous for c.451C>T mutation in Cln1 gene recapitulate INCL phenotype.
Volume: 1
Issue: 12
Pages: 1006-23
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Publication
31578378 | J:280337
First Author: Atiskova Y
Year: 2019
Journal: Sci Rep
Title: Mice deficient in the lysosomal enzyme palmitoyl-protein thioesterase 1 (PPT1) display a complex retinal phenotype.
Volume: 9
Issue: 1
Pages: 14185
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Publication
35358180 | J:324020
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
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Publication
28334871 | J:242242
First Author: Segal-Salto M
Year: 2017
Journal: Hum Mol Genet
Title: Proteomics insights into infantile neuronal ceroid lipofuscinosis (CLN1) point to the involvement of cilia pathology in the disease.
Volume: 26
Issue: 9
Pages: 1678
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Publication
18021406 | J:355837
 
First Author: Qiao X
Year: 2007
Journal: BMC Neurosci
Title: Gene expression profiling in a mouse model of infantile neuronal ceroid lipofuscinosis reveals upregulation of immediate early genes and mediators of the inflammatory response.
Volume: 8
Pages: 95
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Publication
31134714 | J:294414
First Author: Zhao W
Year: 2019
Journal: Mol Reprod Dev
Title: Functional importance of palmitoyl protein thioesterase 1 (PPT1) expression by Sertoli cells in mediating cholesterol metabolism and maintenance of sperm quality.
Volume: 86
Issue: 8
Pages: 984-998
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Publication
12483688 | J:112161
First Author: Ahtiainen L
Year: 2003
Journal: J Comp Neurol
Title: Palmitoyl protein thioesterase 1 is targeted to the axons in neurons.
Volume: 455
Issue: 3
Pages: 368-77
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Publication
16571600 | J:109538
First Author: Kim SJ
Year: 2006
Journal: Hum Mol Genet
Title: Palmitoyl-protein thioesterase-1 deficiency leads to the activation of caspase-9 and contributes to rapid neurodegeneration in INCL.
Volume: 15
Issue: 10
Pages: 1580-6
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Publication
16368712 | J:104521
First Author: Zhang Z
Year: 2006
Journal: Hum Mol Genet
Title: Palmitoyl-protein thioesterase-1 deficiency mediates the activation of the unfolded protein response and neuronal apoptosis in INCL.
Volume: 15
Issue: 2
Pages: 337-46
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Publication
22704978 | J:188057
First Author: Hu J
Year: 2012
Journal: Mol Genet Metab
Title: Intravenous high-dose enzyme replacement therapy with recombinant palmitoyl-protein thioesterase reduces visceral lysosomal storage and modestly prolongs survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis.
Volume: 107
Issue: 1-2
Pages: 213-21
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Publication
18704195 | J:140851
First Author: Kim SJ
Year: 2008
Journal: J Clin Invest
Title: Palmitoyl protein thioesterase-1 deficiency impairs synaptic vesicle recycling at nerve terminals, contributing to neuropathology in humans and mice.
Volume: 118
Issue: 9
Pages: 3075-86
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Publication
28673981 | J:244958
First Author: Shyng C
Year: 2017
Journal: Proc Natl Acad Sci U S A
Title: Synergistic effects of treating the spinal cord and brain in CLN1 disease.
Volume: 114
Issue: 29
Pages: E5920-E5929
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Publication
25205113 | J:213552
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
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Publication
38169466 | J:345002
First Author: Ni H
Year: 2024
Journal: Nat Commun
Title: Cyclical palmitoylation regulates TLR9 signalling and systemic autoimmunity in mice.
Volume: 15
Issue: 1
Pages: 1
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Publication
30089511 | J:317332
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
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Publication
23139814 | J:194798
First Author: Khaibullina A
Year: 2012
Journal: PLoS One
Title: In a model of Batten disease, palmitoyl protein thioesterase-1 deficiency is associated with brown adipose tissue and thermoregulation abnormalities.
Volume: 7
Issue: 11
Pages: e48733
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Publication
38211816 | J:350098
First Author: Bagh MB
Year: 2024
Journal: J Biol Chem
Title: Disruption of lysosomal nutrient sensing scaffold contributes to pathogenesis of a fatal neurodegenerative lysosomal storage disease.
Volume: 300
Issue: 2
Pages: 105641
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Publication
16542649 | J:111503
First Author: Ahtiainen L
Year: 2006
Journal: Exp Cell Res
Title: Palmitoyl protein thioesterase 1 (PPT1) deficiency causes endocytic defects connected to abnormal saposin processing.
Volume: 312
Issue: 9
Pages: 1540-53
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Publication
30946007 | J:274689
   
First Author: Koster KP
Year: 2019
Journal: Elife
Title: Developmental NMDA receptor dysregulation in the infantile neuronal ceroid lipofuscinosis mouse model.
Volume: 8
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Publication
16242638 | J:102821
First Author: Virmani T
Year: 2005
Journal: Neurobiol Dis
Title: Progressively reduced synaptic vesicle pool size in cultured neurons derived from neuronal ceroid lipofuscinosis-1 knockout mice.
Volume: 20
Issue: 2
Pages: 314-23
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Publication
33753498 | J:303993
 
First Author: Yuan W
Year: 2021
Journal: Proc Natl Acad Sci U S A
Title: GFAP hyperpalmitoylation exacerbates astrogliosis and neurodegenerative pathology in PPT1-deficient mice.
Volume: 118
Issue: 13
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Publication
19345705 | J:153490
First Author: Tardy C
Year: 2009
Journal: Biochim Biophys Acta
Title: Palmitoyl protein thioesterase 1 modulates tumor necrosis factor alpha-induced apoptosis.
Volume: 1793
Issue: 7
Pages: 1250-8
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Publication
22031903 | J:177265
First Author: Macauley SL
Year: 2011
Journal: J Neurosci
Title: The role of attenuated astrocyte activation in infantile neuronal ceroid lipofuscinosis.
Volume: 31
Issue: 43
Pages: 15575-85
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Publication
32279353 | J:345946
First Author: Sarkar C
Year: 2020
Journal: J Inherit Metab Dis
Title: Cln1-mutations suppress Rab7-RILP interaction and impair autophagy contributing to neuropathology in a mouse model of infantile neuronal ceroid lipofuscinosis.
Volume: 43
Issue: 5
Pages: 1082-1101
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Publication
33796852 | J:311484
First Author: Perez-Canamas A
Year: 2021
Journal: Brain Commun
Title: Fronto-temporal dementia risk gene TMEM106B has opposing effects in different lysosomal storage disorders.
Volume: 3
Issue: 1
Pages: fcaa200
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Publication
19941651 | J:235261
 
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
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Publication
15647513 | J:112499
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
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Protein Coding Gene
MGP_CAROLIEiJ_G0026424 | -
Type: protein_coding_gene
Organism: Mus caroli
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Protein Coding Gene
MGP_129S1SvImJ_G0028727 | -
Type: protein_coding_gene
Organism: mouse, laboratory
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Protein Coding Gene
MGP_AJ_G0028686 | -
Type: protein_coding_gene
Organism: mouse, laboratory
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Protein Coding Gene
MGP_AKRJ_G0028640 | -
Type: protein_coding_gene
Organism: mouse, laboratory
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Protein Coding Gene
MGP_BALBcJ_G0028709 | -
Type: protein_coding_gene
Organism: mouse, laboratory
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Protein Coding Gene
MGP_C3HHeJ_G0028424 | -
Type: protein_coding_gene
Organism: mouse, laboratory
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Protein Coding Gene
MGI_C57BL6J_1298204 | -
Type: protein_coding_gene
Organism: mouse, laboratory
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Protein Coding Gene
MGP_C57BL6NJ_G0029151 | -
Type: protein_coding_gene
Organism: mouse, laboratory
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Protein Coding Gene
MGP_CASTEiJ_G0027856 | -
Type: protein_coding_gene
Organism: mouse, laboratory
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Protein Coding Gene
MGP_CBAJ_G0028391 | -
Type: protein_coding_gene
Organism: mouse, laboratory
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Protein Coding Gene
MGP_DBA2J_G0028538 | -
Type: protein_coding_gene
Organism: mouse, laboratory
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Protein Coding Gene
MGP_FVBNJ_G0028505 | -
Type: protein_coding_gene
Organism: mouse, laboratory
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Protein Coding Gene
MGP_LPJ_G0028639 | -
Type: protein_coding_gene
Organism: mouse, laboratory
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Protein Coding Gene
MGP_NODShiLtJ_G0028533 | -
Type: protein_coding_gene
Organism: mouse, laboratory
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Protein Coding Gene
MGP_NZOHlLtJ_G0029188 | -
Type: protein_coding_gene
Organism: mouse, laboratory
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Protein Coding Gene
MGP_PWKPhJ_G0027578 | -
Type: protein_coding_gene
Organism: mouse, laboratory
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Protein Coding Gene
MGP_WSBEiJ_G0027936 | -
Type: protein_coding_gene
Organism: mouse, laboratory
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Protein Coding Gene
MGP_PahariEiJ_G0028754 | -
Type: protein_coding_gene
Organism: Mus pahari
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Protein Coding Gene
MGP_SPRETEiJ_G0027404 | -
Type: protein_coding_gene
Organism: Mus spretus
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Publication
10231585 | J:54950
First Author: Zhang Z
Year: 1999
Journal: Gene
Title: Palmitoyl-protein thioesterase gene expression in the developing mouse brain and retina: implications for early loss of vision in infantile neuronal ceroid lipofuscinosis.
Volume: 231
Issue: 1-2
Pages: 203-11
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Publication
35789177 | J:333541
First Author: Dearborn JT
Year: 2022
Journal: Sci Rep
Title: Effects of chronic cannabidiol in a mouse model of naturally occurring neuroinflammation, neurodegeneration, and spontaneous seizures.
Volume: 12
Issue: 1
Pages: 11286
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Publication
36876653 | J:334900
First Author: Peviani M
Year: 2023
Journal: EMBO Mol Med
Title: An innovative hematopoietic stem cell gene therapy approach benefits CLN1 disease in the mouse model.
Volume: 15
Issue: 4
Pages: e15968
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Publication
15193291 | J:91134
First Author: Bible E
Year: 2004
Journal: Neurobiol Dis
Title: Regional and cellular neuropathology in the palmitoyl protein thioesterase-1 null mutant mouse model of infantile neuronal ceroid lipofuscinosis.
Volume: 16
Issue: 2
Pages: 346-59
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Publication
30918483 | J:298787
 
First Author: Sapir T
Year: 2019
Journal: Front Cell Neurosci
Title: The Interactome of Palmitoyl-Protein Thioesterase 1 (PPT1) Affects Neuronal Morphology and Function.
Volume: 13
Pages: 92
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Publication
22331300 | J:183802
First Author: Saha A
Year: 2012
Journal: Hum Mol Genet
Title: The blood-brain barrier is disrupted in a mouse model of infantile neuronal ceroid lipofuscinosis: amelioration by resveratrol.
Volume: 21
Issue: 10
Pages: 2233-44
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Publication
33115477 | J:337601
First Author: Berve K
Year: 2020
Journal: J Neuroinflammation
Title: Sex- and region-biased depletion of microglia/macrophages attenuates CLN1 disease in mice.
Volume: 17
Issue: 1
Pages: 323
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Publication
19416667 | J:232392
First Author: Macauley SL
Year: 2009
Journal: Exp Neurol
Title: Cerebellar pathology and motor deficits in the palmitoyl protein thioesterase 1-deficient mouse.
Volume: 217
Issue: 1
Pages: 124-35
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Publication
32938982 | J:296167
First Author: Nelvagal HR
Year: 2020
Journal: Sci Rep
Title: Comparative proteomic profiling reveals mechanisms for early spinal cord vulnerability in CLN1 disease.
Volume: 10
Issue: 1
Pages: 15157
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Publication
24014511 | J:221698
First Author: Finn R
Year: 2013
Journal: J Child Neurol
Title: Treatment of the Ppt1(-/-) mouse model of infantile neuronal ceroid lipofuscinosis with the N-methyl-D-aspartate (NMDA) receptor antagonist memantine.
Volume: 28
Issue: 9
Pages: 1159-68
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Publication
26238334 | J:274072
 
First Author: Dearborn JT
Year: 2015
Journal: Sci Rep
Title: Comprehensive functional characterization of murine infantile Batten disease including Parkinson-like behavior and dopaminergic markers.
Volume: 5
Pages: 12752
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Publication
28266544 | J:244170
 
First Author: Bagh MB
Year: 2017
Journal: Nat Commun
Title: Misrouting of v-ATPase subunit V0a1 dysregulates lysosomal acidification in a neurodegenerative lysosomal storage disease model.
Volume: 8
Pages: 14612
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Publication
25253854 | J:216609
First Author: Macauley SL
Year: 2014
Journal: J Neurosci
Title: An anti-neuroinflammatory that targets dysregulated glia enhances the efficacy of CNS-directed gene therapy in murine infantile neuronal ceroid lipofuscinosis.
Volume: 34
Issue: 39
Pages: 13077-82
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Publication
34325129 | J:321706
 
First Author: Sadhukhan T
Year: 2021
Journal: Biochem Biophys Res Commun
Title: Ablation of microRNA-155 and neuroinflammation in a mouse model of CLN1-disease.
Volume: 571
Pages: 137-144
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Publication
17656100 | J:134820
First Author: Ahtiainen L
Year: 2007
Journal: Neurobiol Dis
Title: Palmitoyl protein thioesterase 1 (Ppt1)-deficient mouse neurons show alterations in cholesterol metabolism and calcium homeostasis prior to synaptic dysfunction.
Volume: 28
Issue: 1
Pages: 52-64
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Publication
10611498 | J:59196
First Author: Isosomppi J
Year: 1999
Journal: Brain Res Dev Brain Res
Title: Developmental expression of palmitoyl protein thioesterase in normal mice.
Volume: 118
Issue: 1-2
Pages: 1-11
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GXD Expression
GXD Expression
 
Probe: MGI:3724893
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Weak
Sex: Not Specified
Emaps: EMAPS:3516422
Pattern: Regionally restricted
Stage: TS22
Assay Id: MGI:5421544
Age: embryonic day 14.5
Image: MH1487; Specimen C1536
Specimen Label: MH1487; Specimen C1536
Detected: true
Specimen Num: 1
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GXD Expression
GXD Expression
     
Probe: MGI:4426104
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:3516422
Stage: TS22
Assay Id: MGI:5422331
Age: embryonic day 14.5
Specimen Label: ES2567; Specimen S973
Detected: false
Specimen Num: 1
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GXD Expression
GXD Expression
     
Probe: MGI:4426104
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1716122
Stage: TS22
Assay Id: MGI:5422331
Age: embryonic day 14.5
Specimen Label: ES2567; Specimen S973
Detected: false
Specimen Num: 1
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GXD Expression
GXD Expression
 
Probe: MGI:3724893
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Strong
Sex: Not Specified
Emaps: EMAPS:1691622
Pattern: Regionally restricted
Stage: TS22
Assay Id: MGI:5421544
Age: embryonic day 14.5
Image: MH1487; Specimen C1536
Specimen Label: MH1487; Specimen C1536
Detected: true
Specimen Num: 1
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