Progressively reduced synaptic vesicle pool size in cultured neurons derived from neuronal ceroid lipofuscinosis-1 knockout mice
about
Exome-sequencing confirms DNAJC5 mutations as cause of adult neuronal ceroid-lipofuscinosisPalmitoyl protein thioesterase-1 deficiency impairs synaptic vesicle recycling at nerve terminals, contributing to neuropathology in humans and miceInteractions of the proteins of neuronal ceroid lipofuscinosis: clues to functionLarge-scale phenotyping of an accurate genetic mouse model of JNCL identifies novel early pathology outside the central nervous systemActivity-Dependent Degradation of Synaptic Vesicle Proteins Requires Rab35 and the ESCRT PathwayGene expression profiling in a mouse model of infantile neuronal ceroid lipofuscinosis reveals upregulation of immediate early genes and mediators of the inflammatory responseIdentifying cellular pathways modulated by Drosophila palmitoyl-protein thioesterase 1 function.Palmitoyl-protein thioesterase 1 deficiency in Drosophila melanogaster causes accumulation of abnormal storage material and reduced life spanSuccessive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis.Genetic modifiers of Drosophila palmitoyl-protein thioesterase 1-induced degeneration.Proteomic Profiling in the Brain of CLN1 Disease Model Reveals Affected Functional Modules.Reversible Cysteine Acylation Regulates the Activity of Human Palmitoyl-Protein Thioesterase 1 (PPT1).The blood-brain barrier is disrupted in a mouse model of infantile neuronal ceroid lipofuscinosis: amelioration by resveratrol.Photoreceptor phagosome processing defects and disturbed autophagy in retinal pigment epithelium of Cln3Δex1-6 mice modelling juvenile neuronal ceroid lipofuscinosis (Batten disease).Molecular neuropathology of the synapse in sheep with CLN5 Batten diseaseSynaptic vesicle reuse and its implications.Suppression of agrin-22 production and synaptic dysfunction in Cln1 (-/-) mice.Thematic review series: lipid posttranslational modifications. Lysosomal metabolism of lipid-modified proteins.Neuronal ceroid lipofuscinosis with DNAJC5/CSPα mutation has PPT1 pathology and exhibit aberrant protein palmitoylation.Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease.Mutations in palmitoyl-protein thioesterase 1 alter exocytosis and endocytosis at synapses in Drosophila larvae.Misrouting of v-ATPase subunit V0a1 dysregulates lysosomal acidification in a neurodegenerative lysosomal storage disease model.Mechanisms underlying synaptic vulnerability and degeneration in neurodegenerative disease.Synaptic failure: The achilles tendon of sphingolipidosesMorphologic and functional correlates of synaptic pathology in the cathepsin D knockout mouse model of congenital neuronal ceroid lipofuscinosis.The Networks of Genes Encoding Palmitoylated Proteins in Axonal and Synaptic Compartments Are Affected in PPT1 Overexpressing Neuronal-Like Cells.Proteomic mapping of differentially vulnerable pre-synaptic populations identifies regulators of neuronal stability in vivoDecreased sensitivity of palmitoyl protein thioesterase 1-deficient neurons to chemical anoxia.GAP43 overexpression and enhanced neurite outgrowth in mucopolysaccharidosis type IIIB cortical neuron cultures.Altered Cerebellar Short-Term Plasticity but No Change in Postsynaptic AMPA-Type Glutamate Receptors in a Mouse Model of Juvenile Batten Disease.Compromised astrocyte function and survival negatively impact neurons in infantile neuronal ceroid lipofuscinosis
P2860
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P2860
Progressively reduced synaptic vesicle pool size in cultured neurons derived from neuronal ceroid lipofuscinosis-1 knockout mice
description
2005 թուականի Նոյեմբերին հրատարակուած գիտական յօդուած
@hyw
2005 թվականի նոյեմբերին հրատարակված գիտական հոդված
@hy
artículu científicu espublizáu en 2005
@ast
im November 2005 veröffentlichter wissenschaftlicher Artikel
@de
scientific journal article
@en
vedecký článok (publikovaný 2005/11/01)
@sk
vědecký článek publikovaný v roce 2005
@cs
wetenschappelijk artikel (gepubliceerd op 2005/11/01)
@nl
наукова стаття, опублікована в листопаді 2005
@uk
مقالة علمية (نشرت في نوفمبر 2005)
@ar
name
Progressively reduced synaptic ...... lipofuscinosis-1 knockout mice
@ast
Progressively reduced synaptic ...... lipofuscinosis-1 knockout mice
@en
Progressively reduced synaptic ...... lipofuscinosis-1 knockout mice
@nl
type
label
Progressively reduced synaptic ...... lipofuscinosis-1 knockout mice
@ast
Progressively reduced synaptic ...... lipofuscinosis-1 knockout mice
@en
Progressively reduced synaptic ...... lipofuscinosis-1 knockout mice
@nl
prefLabel
Progressively reduced synaptic ...... lipofuscinosis-1 knockout mice
@ast
Progressively reduced synaptic ...... lipofuscinosis-1 knockout mice
@en
Progressively reduced synaptic ...... lipofuscinosis-1 knockout mice
@nl
P2093
P921
P1476
Progressively reduced synaptic ...... lipofuscinosis-1 knockout mice
@en
P2093
Praveena Gupta
Sandra L. Hofmann
Xinran Liu
P304
P356
10.1016/J.NBD.2005.03.012
P577
2005-11-01T00:00:00Z