Palmitoyl protein thioesterase (PPT) localizes into synaptosomes and synaptic vesicles in neurons: implications for infantile neuronal ceroid lipofuscinosis (INCL)
about
Exome-sequencing confirms DNAJC5 mutations as cause of adult neuronal ceroid-lipofuscinosisGlycosylation, transport, and complex formation of palmitoyl protein thioesterase 1 (PPT1)--distinct characteristics in neuronsNeuronal ceroid lipofuscinoses are connected at molecular level: interaction of CLN5 protein with CLN2 and CLN3Palmitoyl protein thioesterase-1 deficiency impairs synaptic vesicle recycling at nerve terminals, contributing to neuropathology in humans and miceThe neuronal ceroid lipofuscinosis Cln8 gene expression is developmentally regulated in mouse brain and up-regulated in the hippocampal kindling model of epilepsy.An over-expression system for characterizing Ppt1 function in DrosophilaNovel interactions of CLN5 support molecular networking between Neuronal Ceroid Lipofuscinosis proteinsInteractions of the proteins of neuronal ceroid lipofuscinosis: clues to functionData on characterizing the gene expression patterns of neuronal ceroid lipofuscinosis genes: CLN1, CLN2, CLN3, CLN5 and their association to interneuron and neurotransmission markers: Parvalbumin and Somatostatin.Gene expression profiling in a mouse model of infantile neuronal ceroid lipofuscinosis reveals upregulation of immediate early genes and mediators of the inflammatory responseBrain gene expression profiles of Cln1 and Cln5 deficient mice unravels common molecular pathways underlying neuronal degeneration in NCL diseases.The Batten disease Palmitoyl Protein Thioesterase 1 gene regulates neural specification and axon connectivity during Drosophila embryonic development.Identification of palmitoyl protein thioesterase 1 in human THP1 monocytes and macrophages and characterization of unique biochemical activities for this enzymeIdentifying cellular pathways modulated by Drosophila palmitoyl-protein thioesterase 1 function.A mutation in canine PPT1 causes early onset neuronal ceroid lipofuscinosis in a Dachshund.The genetic spectrum of human neuronal ceroid-lipofuscinoses.A systematic analysis of protein palmitoylation in Caenorhabditis elegansPalmitoyl-protein thioesterase 1 deficiency in Drosophila melanogaster causes accumulation of abnormal storage material and reduced life spanThe neuronal ceroid-lipofuscinoses.The intracellular location and function of proteins of neuronal ceroid lipofuscinoses.Selectivity and types of cell death in the neuronal ceroid lipofuscinoses.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).Neuronal ceroid lipofuscinosis with DNAJC5/CSPα mutation has PPT1 pathology and exhibit aberrant protein palmitoylation.Lysosomal serine protease CLN2 regulates tumor necrosis factor-alpha-mediated apoptosis in a Bid-dependent mannerMolecular 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.Neuronal ceroid lipofuscinosis: impact of recent genetic advances and expansion of the clinicopathologic spectrum.Palmitoyl-protein thioesterase 1 (PPT1): an obesity-induced rat testicular marker of reduced fertility.Altered glutamate receptor function in the cerebellum of the Ppt1-/- mouse, a murine model of infantile neuronal ceroid lipofuscinosisThe Networks of Genes Encoding Palmitoylated Proteins in Axonal and Synaptic Compartments Are Affected in PPT1 Overexpressing Neuronal-Like Cells.Proteomics insights into infantile neuronal ceroid lipofuscinosis (CLN1) point to the involvement of cilia pathology in the disease.
P2860
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P2860
Palmitoyl protein thioesterase (PPT) localizes into synaptosomes and synaptic vesicles in neurons: implications for infantile neuronal ceroid lipofuscinosis (INCL)
description
2001 nî lūn-bûn
@nan
2001 թուականի Յունուարին հրատարակուած գիտական յօդուած
@hyw
2001 թվականի հունվարին հրատարակված գիտական հոդված
@hy
2001年の論文
@ja
2001年論文
@yue
2001年論文
@zh-hant
2001年論文
@zh-hk
2001年論文
@zh-mo
2001年論文
@zh-tw
2001年论文
@wuu
name
Palmitoyl protein thioesterase ...... l ceroid lipofuscinosis (INCL)
@ast
Palmitoyl protein thioesterase ...... l ceroid lipofuscinosis (INCL)
@en
Palmitoyl protein thioesterase ...... l ceroid lipofuscinosis (INCL)
@en-gb
Palmitoyl protein thioesterase ...... l ceroid lipofuscinosis (INCL)
@nl
type
label
Palmitoyl protein thioesterase ...... l ceroid lipofuscinosis (INCL)
@ast
Palmitoyl protein thioesterase ...... l ceroid lipofuscinosis (INCL)
@en
Palmitoyl protein thioesterase ...... l ceroid lipofuscinosis (INCL)
@en-gb
Palmitoyl protein thioesterase ...... l ceroid lipofuscinosis (INCL)
@nl
prefLabel
Palmitoyl protein thioesterase ...... l ceroid lipofuscinosis (INCL)
@ast
Palmitoyl protein thioesterase ...... l ceroid lipofuscinosis (INCL)
@en
Palmitoyl protein thioesterase ...... l ceroid lipofuscinosis (INCL)
@en-gb
Palmitoyl protein thioesterase ...... l ceroid lipofuscinosis (INCL)
@nl
P2093
P921
P3181
P356
P1476
Palmitoyl protein thioesterase ...... l ceroid lipofuscinosis (INCL)
@en
P2093
E L Eskelinen
M Lehtovirta
O Heinonen
P3181
P356
10.1093/HMG/10.1.69
P407
P577
2001-01-01T00:00:00Z