Cargando…

Sensorimotor and Neurocognitive Dysfunctions Parallel Early Telencephalic Neuropathology in Fucosidosis Mice

Fucosidosis is a lysosomal storage disorder (LSD) caused by lysosomal α-L-fucosidase deficiency. Insufficient α-L-fucosidase activity triggers accumulation of undegraded, fucosylated glycoproteins and glycolipids in various tissues. The human phenotype is heterogeneous, but progressive motor and cog...

Descripción completa

Detalles Bibliográficos
Autores principales:	Stroobants, Stijn, Wolf, Heike, Callaerts-Vegh, Zsuzsanna, Dierks, Thomas, Lübke, Torben, D’Hooge, Rudi
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Frontiers Media S.A. 2018
Materias:	Neuroscience
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5906539/ https://www.ncbi.nlm.nih.gov/pubmed/29706874 http://dx.doi.org/10.3389/fnbeh.2018.00069

Ejemplares similares

A mouse model for fucosidosis recapitulates storage pathology and neurological features of the milder form of the human disease
por: Wolf, Heike, et al.
Publicado: (2016)

Functional Dissociation of Group III Metabotropic Glutamate Receptors Revealed by Direct Comparison between the Behavioral Profiles of Knockout Mouse Lines
por: Goddyn, Hannelore, et al.
Publicado: (2015)

Unpredictable chronic mild stress differentially impairs social and contextual discrimination learning in two inbred mouse strains
por: van Boxelaere, Michiel, et al.
Publicado: (2017)

Impaired Reversal Learning in APPPS1-21 Mice in the Touchscreen Visual Discrimination Task
por: Van den Broeck, Lore, et al.
Publicado: (2019)

Aged Tmem106b knockout mice display gait deficits in coincidence with Purkinje cell loss and only limited signs of non‐motor dysfunction
por: Stroobants, Stijn, et al.
Publicado: (2020)

Stimulus generalization and return of fear in C57BL/6J mice
por: Boddez, Yannick, et al.
Publicado: (2012)

Nxf7 deficiency impairs social exploration and spatio-cognitive abilities as well as hippocampal synaptic plasticity in mice
por: Callaerts-Vegh, Zsuzsanna, et al.
Publicado: (2015)

Neuronal Dysfunction and Behavioral Abnormalities Are Evoked by Neural Cells and Aggravated by Inflammatory Microglia in Peroxisomal β-Oxidation Deficiency
por: Beckers, Lien, et al.
Publicado: (2018)

Post-weaning infant-to-mother bonding in nutritionally independent female mice
por: Stroobants, Stijn, et al.
Publicado: (2020)

Increased Insoluble Amyloid-β Induces Negligible Cognitive Deficits in Old App(NL/NL) Knock-In Mice
por: Salas, Isabel H., et al.
Publicado: (2018)

Chronic enzyme replacement therapy ameliorates neuropathology in alpha‐mannosidosis mice
por: Damme, Markus, et al.
Publicado: (2015)

Siblings with fucosidosis
por: Muthusamy, Karthik, et al.
Publicado: (2014)

Quinolinic acid injection in mouse medial prefrontal cortex affects reversal learning abilities, cortical connectivity and hippocampal synaptic plasticity
por: Latif-Hernandez, Amira, et al.
Publicado: (2016)

MEK inhibition ameliorates social behavior phenotypes in a Spred1 knockout mouse model for RASopathy disorders
por: Borrie, Sarah C., et al.
Publicado: (2021)

The effects of intracisternal enzyme replacement versus sham treatment on central neuropathology in preclinical canine fucosidosis
por: Kondagari, Gauthami Sudhamayee, et al.
Publicado: (2015)

Spatial reversal learning defect coincides with hypersynchronous telencephalic BOLD functional connectivity in APP(NL-F/NL-F) knock-in mice
por: Shah, Disha, et al.
Publicado: (2018)

Correction: Tetraspanin 6: A novel regulator of hippocampal synaptic transmission and long term plasticity
por: Salas, Isabel H., et al.
Publicado: (2017)

Deregulation of neuronal miRNAs induced by amyloid-β or TAU pathology
por: Sierksma, Annerieke, et al.
Publicado: (2018)

Tetraspanin 6: A novel regulator of hippocampal synaptic transmission and long term plasticity
por: Salas, Isabel H., et al.
Publicado: (2017)

Correction: Tetraspanin 6: A novel regulator of hippocampal synaptic transmission and long term plasticity
por: Salas, Isabel H., et al.
Publicado: (2017)

A20/TNFAIP3 heterozygosity predisposes to behavioral symptoms in a mouse model for neuropsychiatric lupus
por: Daems, C., et al.
Publicado: (2019)

Generation and Characterization of an Nxf7 Knockout Mouse to Study NXF5 Deficiency in a Patient with Intellectual Disability
por: Vanmarsenille, Lieselot, et al.
Publicado: (2013)

Microglia lacking a peroxisomal β-oxidation enzyme chronically alter their inflammatory profile without evoking neuronal and behavioral deficits
por: Beckers, Lien, et al.
Publicado: (2019)

An unusual presentation of fucosidosis in a Chinese boy: a case report and literature review (childhood fucosidosis)
por: Mao, Shao-Jia, et al.
Publicado: (2022)

Ocular findings in a patient with fucosidosis
por: Sánchez, Lucía Rivera, et al.
Publicado: (2016)

Fucosidosis mimicking juvenile idiopathic arthritis
por: Wynne, Emma, et al.
Publicado: (2018)

Folic Acid Fortification Prevents Morphological and Behavioral Consequences of X-Ray Exposure During Neurulation
por: Craenen, Kai, et al.
Publicado: (2021)

LAMP-2 deficiency leads to hippocampal dysfunction but normal clearance of neuronal substrates of chaperone-mediated autophagy in a mouse model for Danon disease
por: Rothaug, Michelle, et al.
Publicado: (2015)

Diagnosis and Supportive Management of Fucosidosis: A Case Report
por: Kaur, Arpanjeet, et al.
Publicado: (2019)

rAAV2/7 vector-mediated overexpression of alpha-synuclein in mouse substantia nigra induces protein aggregation and progressive dose-dependent neurodegeneration
por: Oliveras-Salvá, Marusela, et al.
Publicado: (2013)

FUCOSIDOSIS: DEFICIENCY OF ALPHA-L-FUCOSIDASE IN CULTURED SKIN FIBROBLASTS
por: Zielke, Klaus, et al.
Publicado: (1972)

Clinical and neuroradiological approach to fucosidosis in a child with atypical presentation
por: Zubarioglu, Tanyel, et al.
Publicado: (2015)

Differential involvement of the extracellular 6-O-endosulfatases Sulf1 and Sulf2 in brain development and neuronal and behavioural plasticity
por: Kalus, Ina, et al.
Publicado: (2009)

EphA4 loss improves social memory performance and alters dendritic spine morphology without changes in amyloid pathology in a mouse model of Alzheimer’s disease
por: Poppe, Lindsay, et al.
Publicado: (2019)

Fucosidosis in a Chinese boy: a case report and literature review
por: Wang, Lingxing, et al.
Publicado: (2020)

Telencephalic regulation of the HPA axis in birds
por: Smulders, Tom V.
Publicado: (2021)

Sensorimotor Learning: Neurocognitive Mechanisms and Individual Differences
por: Seidler, R. D., et al.
Publicado: (2017)

Sensorimotor Synchronization in Healthy Aging and Neurocognitive Disorders
por: von Schnehen, Andres, et al.
Publicado: (2022)

The Early Phase of Vascularization in Intraocular Telencephalic Transplants
por: Tuba, Andrea, et al.
Publicado: (1997)

Telencephalic Projections of the Midline and Intralaminar Nuclei in the Cat
por: Mckegney, F. Patrick
Publicado: (1958)

Cannot write session to /tmp/vufind_sessions/sess_tpddkmg2eckpcj1h9nv06g7tsr