Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis.
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Moving towards effective therapeutic strategies for Neuronal Ceroid LipofuscinosisA murine model of variant late infantile ceroid lipofuscinosis recapitulates behavioral and pathological phenotypes of human diseaseInteractions of the proteins of neuronal ceroid lipofuscinosis: clues to functionAbnormally increased surface expression of AMPA receptors in the cerebellum, cortex and striatum of Cln3(-/-) miceCerebellar alterations and gait defects as therapeutic outcome measures for enzyme replacement therapy in α-mannosidosis.Multi-system disorders of glycosphingolipid and ganglioside metabolismA novel interaction of CLN3 with nonmuscle myosin-IIB and defects in cell motility of Cln3(-/-) cellsImmunosuppression alters disease severity in juvenile Batten disease miceAn anti-neuroinflammatory that targets dysregulated glia enhances the efficacy of CNS-directed gene therapy in murine infantile neuronal ceroid lipofuscinosisAge-dependent therapeutic effect of memantine in a mouse model of juvenile Batten diseaseNeurologic abnormalities in mouse models of the lysosomal storage disorders mucolipidosis II and mucolipidosis III γ.Evidence for aberrant astrocyte hemichannel activity in Juvenile Neuronal Ceroid Lipofuscinosis (JNCL).Altered sensitivity of cerebellar granule cells to glutamate receptor overactivation in the Cln3(Δex7/8)-knock-in mouse model of juvenile neuronal ceroid lipofuscinosis.Temporary inhibition of AMPA receptors induces a prolonged improvement of motor performance in a mouse model of juvenile Batten diseaseFinding the most appropriate mouse model of juvenile CLN3 (Batten) disease for therapeutic studies: the importance of genetic background and gender.α-Synuclein-independent histopathological and motor deficits in mice lacking the endolysosomal Parkinsonism protein Atp13a2.Lithium rescues the impaired autophagy process in CbCln3(Δex7/8/Δex7/8) cerebellar cells and reduces neuronal vulnerability to cell death via IMPase inhibition.Altered biometal homeostasis is associated with CLN6 mRNA loss in mouse neuronal ceroid lipofuscinosisLocation- and sex-specific differences in weight and motor coordination in two commonly used mouse strains.Antigen presenting cell abnormalities in the Cln3(-/-) mouse model of juvenile neuronal ceroid lipofuscinosisLoss of Cln5 causes altered neurogenesis in a mouse model of a childhood neurodegenerative disorder.A zebrafish model of CLN2 disease is deficient in tripeptidyl peptidase 1 and displays progressive neurodegeneration accompanied by a reduction in proliferation.Defective synaptic transmission causes disease signs in a mouse model of juvenile neuronal ceroid lipofuscinosis.A multi-ingredient dietary supplement abolishes large-scale brain cell loss, improves sensory function, and prevents neuronal atrophy in aging mice.Deregulation of biometal homeostasis: the missing link for neuronal ceroid lipofuscinoses?c-Jun is essential for the induction of Il-1β gene expression in in vitro activated Bergmann glial cells.Altered Cerebellar Short-Term Plasticity but No Change in Postsynaptic AMPA-Type Glutamate Receptors in a Mouse Model of Juvenile Batten Disease.
P2860
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P2860
Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis.
description
2009 nî lūn-bûn
@nan
2009 թուականի Փետրուարին հրատարակուած գիտական յօդուած
@hyw
2009 թվականի փետրվարին հրատարակված գիտական հոդված
@hy
2009年の論文
@ja
2009年論文
@yue
2009年論文
@zh-hant
2009年論文
@zh-hk
2009年論文
@zh-mo
2009年論文
@zh-tw
2009年论文
@wuu
name
Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis.
@ast
Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis.
@en
type
label
Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis.
@ast
Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis.
@en
prefLabel
Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis.
@ast
Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis.
@en
P2093
P2860
P1433
P1476
Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis
@en
P2093
Amanda L Getty
Charlie C Pontikis
David A Pearce
Jared W Benedict
Jill M Weimer
Ming J Lim
P2860
P304
P356
10.1016/J.BRAINRES.2009.02.009
P407
P577
2009-02-20T00:00:00Z