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Search results 1 to 100 out of 160 for Hexb

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Category: Publication
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Type Details Score
Publication
31547903 | J:301674
 
Year: 2019
Journal: J Am Assoc Lab Anim Sci
Title: Abstracts of Scientific Presentations 2019 AALAS National Meeting.
Volume: 58
Issue: 5
Pages: 607-726
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Publication
36977586 | J:335184
First Author: Eichler A
Year: 2023
Journal: J Neurosci
Title: Microglial Cytokines Mediate Plasticity Induced by 10 Hz Repetitive Magnetic Stimulation.
Volume: 43
Issue: 17
Pages: 3042-3060
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Publication
23028353 | J:190450
First Author: Sargeant TJ
Year: 2012
Journal: PLoS Genet
Title: Characterization of inducible models of Tay-Sachs and related disease.
Volume: 8
Issue: 9
Pages: e1002943
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Publication
32541832 | J:306642
First Author: Masuda T
Year: 2020
Journal: Nat Immunol
Title: Novel Hexb-based tools for studying microglia in the CNS.
Volume: 21
Issue: 7
Pages: 802-815
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Publication
7550345 | J:29268
First Author: Sango K
Year: 1995
Journal: Nat Genet
Title: Mouse models of Tay-Sachs and Sandhoff diseases differ in neurologic phenotype and ganglioside metabolism.
Volume: 11
Issue: 2
Pages: 170-6
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Publication
7918686 | J:21159
First Author: Triggs-Raine BL
Year: 1994
Journal: Biochim Biophys Acta
Title: Characterization of the murine beta-hexosaminidase (HEXB) gene.
Volume: 1227
Issue: 1-2
Pages: 79-86
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Publication
8789434 | J:30899
First Author: Phaneuf D
Year: 1996
Journal: Hum Mol Genet
Title: Dramatically different phenotypes in mouse models of human Tay-Sachs and Sandhoff diseases.
Volume: 5
Issue: 1
Pages: 1-14
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Publication
19603551 | J:310104
First Author: Terashima T
Year: 2009
Journal: J Clin Invest
Title: DRG-targeted helper-dependent adenoviruses mediate selective gene delivery for therapeutic rescue of sensory neuronopathies in mice.
Volume: 119
Issue: 7
Pages: 2100-112
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Publication
28575132 | J:250318
First Author: Ogawa Y
Year: 2017
Journal: PLoS One
Title: Abnormal differentiation of Sandhoff disease model mouse-derived multipotent stem cells toward a neural lineage.
Volume: 12
Issue: 6
Pages: e0178978
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Publication
23383290 | J:195997
First Author: Ogawa Y
Year: 2013
Journal: PLoS One
Title: Impaired neural differentiation of induced pluripotent stem cells generated from a mouse model of Sandhoff disease.
Volume: 8
Issue: 1
Pages: e55856
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Publication
10339597 | J:55198
First Author: Jeyakumar M
Year: 1999
Journal: Proc Natl Acad Sci U S A
Title: Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N-butyldeoxynojirimycin.
Volume: 96
Issue: 11
Pages: 6388-93
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Publication
15198669 | J:91444
First Author: Buccoliero R
Year: 2004
Journal: J Neurochem
Title: Phospholipid synthesis is decreased in neuronal tissue in a mouse model of Sandhoff disease.
Volume: 90
Issue: 1
Pages: 80-8
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Publication
29325092 | J:260516
First Author: Niemir N
Year: 2018
Journal: Hum Mol Genet
Title: Intravenous administration of scAAV9-Hexb normalizes lifespan and prevents pathology in Sandhoff disease mice.
Volume: 27
Issue: 6
Pages: 954-968
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Publication
12617783 | J:81864
First Author: Juneja SC
Year: 2002
Journal: Reprod Fertil Dev
Title: Development of infertility at young adult age in a mouse model of human Sandhoff disease.
Volume: 14
Issue: 7-8
Pages: 407-12
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Publication
7959736 | J:18931
First Author: Yamanaka S
Year: 1994
Journal: Genomics
Title: Structure and expression of the mouse beta-hexosaminidase genes, Hexa and Hexb.
Volume: 21
Issue: 3
Pages: 588-96
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Publication
14722612 | J:87617
First Author: Yamaguchi A
Year: 2004
Journal: J Clin Invest
Title: Possible role of autoantibodies in the pathophysiology of GM2 gangliosidoses.
Volume: 113
Issue: 2
Pages: 200-8
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Publication
28084424 | J:287686
 
First Author: Ogawa Y
Year: 2017
Journal: Sci Rep
Title: FcRγ-dependent immune activation initiates astrogliosis during the asymptomatic phase of Sandhoff disease model mice.
Volume: 7
Pages: 40518
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Publication
8896570 | J:36305
First Author: Sango K
Year: 1996
Journal: Nat Genet
Title: Mice lacking both subunits of lysosomal beta-hexosaminidase display gangliosidosis and mucopolysaccharidosis.
Volume: 14
Issue: 3
Pages: 348-52
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Publication
30026035 | J:268456
 
First Author: Ogawa Y
Year: 2018
Journal: Neurobiol Dis
Title: Inhibition of astrocytic adenosine receptor A2A attenuates microglial activation in a mouse model of Sandhoff disease.
Volume: 118
Pages: 142-154
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Publication
22496568 | J:184450
First Author: Keilani S
Year: 2012
Journal: J Neurosci
Title: Lysosomal dysfunction in a mouse model of Sandhoff disease leads to accumulation of ganglioside-bound amyloid-β peptide.
Volume: 32
Issue: 15
Pages: 5223-36
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Publication
8634145 | J:31304
First Author: Norflus F
Year: 1996
Journal: DNA Cell Biol
Title: Promoters for the human beta-hexosaminidase genes, HEXA and HEXB.
Volume: 15
Issue: 2
Pages: 89-97
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Publication
9645704 | J:48608
First Author: Trasler J
Year: 1998
Journal: Endocrinology
Title: Characterization of the testis and epididymis in mouse models of human Tay Sachs and Sandhoff diseases and partial determination of accumulated gangliosides.
Volume: 139
Issue: 7
Pages: 3280-8
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Publication
9223328 | J:41950
First Author: Liu Y
Year: 1997
Journal: Proc Natl Acad Sci U S A
Title: Mouse model of GM2 activator deficiency manifests cerebellar pathology and motor impairment.
Volume: 94
Issue: 15
Pages: 8138-43
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Publication
31340161 | J:282790
     
First Author: Ogawa Y
Year: 2019
Journal: Neurosci Res
Title: Abnormal organization during neurodevelopment in a mouse model of Sandhoff disease.
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Publication
10591618 | J:59829
First Author: Adamali HI
Year: 1999
Journal: J Androl
Title: I. Abnormalities in cells of the testis, efferent ducts, and epididymis in juvenile and adult mice with beta-hexosaminidase A and B deficiency.
Volume: 20
Issue: 6
Pages: 779-802
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Publication
35357926 | J:323822
First Author: Kohno K
Year: 2022
Journal: Science
Title: A spinal microglia population involved in remitting and relapsing neuropathic pain.
Volume: 376
Issue: 6588
Pages: 86-90
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Publication
15748167 | J:96874
First Author: Tsuji D
Year: 2005
Journal: J Neurochem
Title: Specific induction of macrophage inflammatory protein 1-alpha in glial cells of Sandhoff disease model mice associated with accumulation of N-acetylhexosaminyl glycoconjugates.
Volume: 92
Issue: 6
Pages: 1497-507
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Publication
33555074 | J:323961
First Author: Wieghofer P
Year: 2021
Journal: EMBO J
Title: Mapping the origin and fate of myeloid cells in distinct compartments of the eye by single-cell profiling.
Volume: 40
Issue: 6
Pages: e105123
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Publication
37635351 | J:339857
First Author: Faust TE
Year: 2023
Journal: Cell Rep
Title: A comparative analysis of microglial inducible Cre lines.
Volume: 42
Issue: 9
Pages: 113031
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Publication
2971567 | J:9381
First Author: Bapat B
Year: 1988
Journal: FEBS Lett
Title: Cloning and sequence analysis of a cDNA encoding the beta-subunit of mouse beta-hexosaminidase.
Volume: 237
Issue: 1-2
Pages: 191-5
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Publication
8253842 | J:302574
First Author: Miller DJ
Year: 1993
Journal: J Cell Biol
Title: Egg cortical granule N-acetylglucosaminidase is required for the mouse zona block to polyspermy.
Volume: 123
Issue: 6 Pt 1
Pages: 1431-40
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Publication
14691112 | J:119632
First Author: Shimizu T
Year: 2004
Journal: J Dent Res
Title: Genetic analysis of crown size in the first molars using SMXA recombinant inbred mouse strains.
Volume: 83
Issue: 1
Pages: 45-9
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Publication
12887594 | J:103900
First Author: Pelled D
Year: 2003
Journal: Neuropathol Appl Neurobiol
Title: Reduced rates of axonal and dendritic growth in embryonic hippocampal neurones cultured from a mouse model of Sandhoff disease.
Volume: 29
Issue: 4
Pages: 341-9
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Publication
16123447 | J:101337
First Author: Sango K
Year: 2005
Journal: Invest Ophthalmol Vis Sci
Title: Impaired neurite outgrowth in the retina of a murine model of Sandhoff disease.
Volume: 46
Issue: 9
Pages: 3420-5
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Publication
11849560 | J:103802
First Author: Sango K
Year: 2002
Journal: Neuropathol Appl Neurobiol
Title: Lysosomal storage results in impaired survival but normal neurite outgrowth in dorsal root ganglion neurones from a mouse model of Sandhoff disease.
Volume: 28
Issue: 1
Pages: 23-34
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Publication
18611415 | J:139219
First Author: Gulinello M
Year: 2008
Journal: Behav Brain Res
Title: Early deficits in motor coordination and cognitive dysfunction in a mouse model of the neurodegenerative lysosomal storage disorder, Sandhoff disease.
Volume: 193
Issue: 2
Pages: 315-9
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Publication
9576752 | J:47677
First Author: Norflus F
Year: 1998
Journal: J Clin Invest
Title: Bone marrow transplantation prolongs life span and ameliorates neurologic manifestations in Sandhoff disease mice.
Volume: 101
Issue: 9
Pages: 1881-8
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Publication
12657883 | J:89803
First Author: Suzuki K
Year: 2003
Journal: Neuroreport
Title: Neuronal accumulation of alpha- and beta-synucleins in the brain of a GM2 gangliosidosis mouse model.
Volume: 14
Issue: 4
Pages: 551-4
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Publication
27018595 | J:234702
First Author: Kitakaze K
Year: 2016
Journal: J Clin Invest
Title: Protease-resistant modified human β-hexosaminidase B ameliorates symptoms in GM2 gangliosidosis model.
Volume: 126
Issue: 5
Pages: 1691-703
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Publication
20374428 | J:163568
First Author: Denny CA
Year: 2010
Journal: J Neurochem
Title: Restricted ketogenic diet enhances the therapeutic action of N-butyldeoxynojirimycin towards brain GM2 accumulation in adult Sandhoff disease mice.
Volume: 113
Issue: 6
Pages: 1525-35
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Publication
21852247 | J:176890
First Author: Sargeant TJ
Year: 2011
Journal: Hum Mol Genet
Title: Adeno-associated virus-mediated expression of β-hexosaminidase prevents neuronal loss in the Sandhoff mouse brain.
Volume: 20
Issue: 22
Pages: 4371-80
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Publication
28377426 | J:243215
First Author: Lecommandeur E
Year: 2017
Journal: J Lipid Res
Title: Alterations in endo-lysosomal function induce similar hepatic lipid profiles in rodent models of drug-induced phospholipidosis and Sandhoff disease.
Volume: 58
Issue: 7
Pages: 1306-1314
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Publication
16888648 | J:132341
First Author: Bifsha P
Year: 2007
Journal: Cell Death Differ
Title: Altered gene expression in cells from patients with lysosomal storage disorders suggests impairment of the ubiquitin pathway.
Volume: 14
Issue: 3
Pages: 511-23
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Publication
8286571 | J:326248
First Author: O'Brien DA
Year: 1993
Journal: Biol Reprod
Title: Mouse Sertoli cells secrete mannose 6-phosphate containing glycoproteins that are endocytosed by spermatogenic cells.
Volume: 49
Issue: 5
Pages: 1055-65
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Publication
8269854 | J:99375
First Author: Miller DJ
Year: 1993
Journal: Development
Title: Sperm require beta-N-acetylglucosaminidase to penetrate through the egg zona pellucida.
Volume: 118
Issue: 4
Pages: 1279-89
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Publication
9562524 | J:47488
First Author: Tifft CJ
Year: 1997
Journal: Ann Med
Title: The beta-hexosaminidase deficiency disorders: development of a clinical paradigm in the mouse.
Volume: 29
Issue: 6
Pages: 557-61
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Publication
17372214 | J:120322
First Author: Speak AO
Year: 2007
Journal: Proc Natl Acad Sci U S A
Title: Implications for invariant natural killer T cell ligands due to the restricted presence of isoglobotrihexosylceramide in mammals.
Volume: 104
Issue: 14
Pages: 5971-6
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Publication
21151498 | J:167279
First Author: García-Faroldi G
Year: 2010
Journal: PLoS One
Title: Polyamines are present in mast cell secretory granules and are important for granule homeostasis.
Volume: 5
Issue: 11
Pages: e15071
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Publication
24057669 | J:204890
First Author: Cachón-González MB
Year: 2014
Journal: Hum Mol Genet
Title: Reversibility of neuropathology in Tay-Sachs-related diseases.
Volume: 23
Issue: 3
Pages: 730-48
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Publication
26545928 | J:228747
 
First Author: Loth MK
Year: 2016
Journal: Neurobiol Dis
Title: TSPO in a murine model of Sandhoff disease: presymptomatic marker of neurodegeneration and disease pathophysiology.
Volume: 85
Pages: 174-186
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Publication
25795792 | J:221872
First Author: Akgoc Z
Year: 2015
Journal: J Lipid Res
Title: Bis(monoacylglycero)phosphate: a secondary storage lipid in the gangliosidoses.
Volume: 56
Issue: 5
Pages: 1006-13
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Publication
17442056 | J:122595
First Author: Denny CA
Year: 2007
Journal: J Neurochem
Title: Neurochemical, morphological, and neurophysiological abnormalities in retinas of Sandhoff and GM1 gangliosidosis mice.
Volume: 101
Issue: 5
Pages: 1294-302
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Publication
35444273 | J:330324
First Author: Masuda T
Year: 2022
Journal: Nature
Title: Specification of CNS macrophage subsets occurs postnatally in defined niches.
Volume: 604
Issue: 7907
Pages: 740-748
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Publication
25015817 | J:317584
First Author: Fukuishi N
Year: 2014
Journal: J Immunol
Title: Does β-hexosaminidase function only as a degranulation indicator in mast cells? The primary role of β-hexosaminidase in mast cell granules.
Volume: 193
Issue: 4
Pages: 1886-94
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Publication
20856892 | J:220947
 
First Author: Kanzaki S
Year: 2010
Journal: PLoS One
Title: Thymic alterations in GM2 gangliosidoses model mice.
Volume: 5
Issue: 8
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Publication
9774663 | J:50358
First Author: Gwynn B
Year: 1998
Journal: Mol Cell Biol
Title: Intracisternal A-particle element transposition into the murine beta-glucuronidase gene correlates with loss of enzyme activity: a new model for beta-glucuronidase deficiency in the C3H mouse.
Volume: 18
Issue: 11
Pages: 6474-81
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Publication
28007910 | J:241437
First Author: Hooper AWM
Year: 2017
Journal: Hum Mol Genet
Title: Neuronal pentraxin 1 depletion delays neurodegeneration and extends life in Sandhoff disease mice.
Volume: 26
Issue: 4
Pages: 661-673
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Publication
9122231 | J:39394
First Author: Ohshima T
Year: 1997
Journal: Proc Natl Acad Sci U S A
Title: alpha-Galactosidase A deficient mice: a model of Fabry disease.
Volume: 94
Issue: 6
Pages: 2540-4
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Publication
26467605 | J:237590
 
First Author: Richardson K
Year: 2016
Journal: Behav Brain Res
Title: Circadian profiling in two mouse models of lysosomal storage disorders; Niemann Pick type-C and Sandhoff disease.
Volume: 297
Pages: 213-23
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Publication
34201771 | J:326926
 
First Author: Kot S
Year: 2021
Journal: Int J Mol Sci
Title: Investigating Immune Responses to the scAAV9-HEXM Gene Therapy Treatment in Tay-Sachs Disease and Sandhoff Disease Mouse Models.
Volume: 22
Issue: 13
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9302266 | J:43447
First Author: Huang JQ
Year: 1997
Journal: Hum Mol Genet
Title: Apoptotic cell death in mouse models of GM2 gangliosidosis and observations on human Tay-Sachs and Sandhoff diseases.
Volume: 6
Issue: 11
Pages: 1879-85
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Publication
11707436 | J:124832
First Author: Hepbildikler ST
Year: 2002
Journal: J Biol Chem
Title: Physiological substrates for human lysosomal beta -hexosaminidase S.
Volume: 277
Issue: 4
Pages: 2562-72
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9184660 | J:41920
First Author: Suzuki K
Year: 1997
Journal: J Neuropathol Exp Neurol
Title: Mice deficient in all forms of lysosomal beta-hexosaminidase show mucopolysaccharidosis-like pathology.
Volume: 56
Issue: 6
Pages: 693-703
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Publication
33219123 | J:299205
First Author: Lee JP
Year: 2020
Journal: Proc Natl Acad Sci U S A
Title: Chemical mutagenesis of a GPCR ligand: Detoxifying "inflammo-attraction" to direct therapeutic stem cell migration.
Volume: 117
Issue: 49
Pages: 31177-31188
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Publication
27453479 | J:236789
First Author: Suzuki K
Year: 2016
Journal: Exp Neurol
Title: Accumulated α-synuclein affects the progression of GM2 gangliosidoses.
Volume: 284
Issue: Pt A
Pages: 38-49
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Publication
19166507 | J:149170
First Author: Martino S
Year: 2009
Journal: J Neurochem
Title: Neural precursor cell cultures from GM2 gangliosidosis animal models recapitulate the biochemical and molecular hallmarks of the brain pathology.
Volume: 109
Issue: 1
Pages: 135-47
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Publication
12756243 | J:84955
First Author: Pelled D
Year: 2003
Journal: J Biol Chem
Title: Inhibition of calcium uptake via the sarco/endoplasmic reticulum Ca2+-ATPase in a mouse model of Sandhoff disease and prevention by treatment with N-butyldeoxynojirimycin.
Volume: 278
Issue: 32
Pages: 29496-501
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Publication
9417048 | J:45044
First Author: Yuziuk JA
Year: 1998
Journal: J Biol Chem
Title: Specificity of mouse GM2 activator protein and beta-N-acetylhexosaminidases A and B. Similarities and differences with their human counterparts in the catabolism of GM2.
Volume: 273
Issue: 1
Pages: 66-72
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6458607 | J:320130
First Author: Kresse H
Year: 1981
Journal: J Biol Chem
Title: Liberation of N-acetylglucosamine-6-sulfate by human beta-N-acetylhexosaminidase A.
Volume: 256
Issue: 24
Pages: 12926-32
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Publication
2947337 | J:8520
First Author: Killary AM
Year: 1986
Journal: Somat Cell Mol Genet
Title: Assignment of genes encoding dihydrofolate reductase and hexosaminidase B to Mus musculus chromosome 13.
Volume: 12
Issue: 6
Pages: 641-8
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Publication
8147878 | J:17353
First Author: Della Fazia MA
Year: 1994
Journal: Biochem Biophys Res Commun
Title: Different expression of beta-N-acetylhexosaminidase in mouse tissues.
Volume: 199
Issue: 3
Pages: 1341-6
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Publication
9237263 | J:42860
First Author: Johnson MD
Year: 1997
Journal: Mol Hum Reprod
Title: Genetic expression of hexokinase and glucose phosphate isomerase in late-stage mouse preimplantation embryos: transcription activities in glucose/phosphate-containing HTF and glucose/phosphate-free P1 media.
Volume: 3
Issue: 4
Pages: 351-7
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Publication
- | J:23181
First Author: Killary AM
Year: 1986
Journal: Am J Hum Genet
Title: Assignment of the genes encoding dihydrofolate reductase and hexosaminidase B to mouse Chromosome 13
Volume: 39
Issue: Suppl
Pages: A159 (Abstr.)
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Publication
10021458 | J:53080
First Author: Liu Y
Year: 1999
Journal: J Clin Invest
Title: A genetic model of substrate deprivation therapy for a glycosphingolipid storage disorder.
Volume: 103
Issue: 4
Pages: 497-505
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Publication
34172992 | J:321681
First Author: Cachón-González MB
Year: 2021
Journal: Hum Mol Genet
Title: Expression of Ripk1 and DAM genes correlates with severity and progression of Krabbe disease.
Volume: 30
Issue: 22
Pages: 2082-2099
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Publication
22451654 | J:185446
First Author: Gushulak L
Year: 2012
Journal: J Biol Chem
Title: Hyaluronidase 1 and β-hexosaminidase have redundant functions in hyaluronan and chondroitin sulfate degradation.
Volume: 287
Issue: 20
Pages: 16689-97
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Publication
15155903 | J:90687
First Author: Wu YP
Year: 2004
Journal: Proc Natl Acad Sci U S A
Title: Deletion of macrophage-inflammatory protein 1 alpha retards neurodegeneration in Sandhoff disease mice.
Volume: 101
Issue: 22
Pages: 8425-30
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Publication
26681805 | J:229309
First Author: Brandenstein L
Year: 2016
Journal: Hum Mol Genet
Title: Lysosomal dysfunction and impaired autophagy in a novel mouse model deficient for the lysosomal membrane protein Cln7.
Volume: 25
Issue: 4
Pages: 777-91
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Publication
35482418 | J:326198
 
First Author: Rehm A
Year: 2022
Journal: JCI Insight
Title: EBAG9 controls CD8+ T cell memory formation responding to tumor challenge in mice.
Volume: 7
Issue: 11
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Publication
27605553 | J:259551
First Author: Kirkegaard T
Year: 2016
Journal: Sci Transl Med
Title: Heat shock protein-based therapy as a potential candidate for treating the sphingolipidoses.
Volume: 8
Issue: 355
Pages: 355ra118
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Publication
23727835 | J:201101
First Author: Abo-Ouf H
Year: 2013
Journal: Hum Mol Genet
Title: Deletion of tumor necrosis factor-α ameliorates neurodegeneration in Sandhoff disease mice.
Volume: 22
Issue: 19
Pages: 3960-75
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Publication
1054503 | J:5526
First Author: Gilbert F
Year: 1975
Journal: Proc Natl Acad Sci U S A
Title: Tay-Sachs' and Sandhoff's diseases: the assignment of genes for hexosaminidase A and B to individual human chromosomes.
Volume: 72
Issue: 1
Pages: 263-7
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Publication
9728335 | J:52311
First Author: Kolter T
Year: 1998
Journal: J Inherit Metab Dis
Title: Glycosphingolipid degradation and animal models of GM2-gangliosidoses.
Volume: 21
Issue: 5
Pages: 548-63
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Publication
8678993 | J:34778
First Author: Xue J
Year: 1996
Journal: Mamm Genome
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