CLN3 protein regulates lysosomal pH and alters intracellular processing of Alzheimer's amyloid-beta protein precursor and cathepsin D in human cells
about
Defective lysosomal arginine transport in juvenile Batten diseaseNeuronal ceroid lipofuscinosis protein CLN3 interacts with motor proteins and modifies location of late endosomal compartmentsMembrane trafficking and mitochondrial abnormalities precede subunit c deposition in a cerebellar cell model of juvenile neuronal ceroid lipofuscinosisVaried mechanisms underlie the free sialic acid storage disordersAlterations in ROS activity and lysosomal pH account for distinct patterns of macroautophagy in LINCL and JNCL fibroblasts.Kinetics of bidirectional H+ and substrate transport by the proton-dependent amino acid symporter PAT1A novel interaction of CLN3 with nonmuscle myosin-IIB and defects in cell motility of Cln3(-/-) cellsThe neuronal ceroid-lipofuscinoses.Genetics of inherited human epilepsiesCurrent state of clinical and morphological features in human NCL.The intracellular location and function of proteins of neuronal ceroid lipofuscinoses.Unbiased Cell-based Screening in a Neuronal Cell Model of Batten Disease Highlights an Interaction between Ca2+ Homeostasis, Autophagy, and CLN3 Protein Function.Lithium rescues the impaired autophagy process in CbCln3(Δex7/8/Δex7/8) cerebellar cells and reduces neuronal vulnerability to cell death via IMPase inhibition.Nitric oxide signaling is disrupted in the yeast model for Batten disease.Host pathways important for Coxiella burnetii infection revealed by genome-wide RNA interference screening.Antigen presenting cell abnormalities in the Cln3(-/-) mouse model of juvenile neuronal ceroid lipofuscinosisOsmoregulation of ceroid neuronal lipofuscinosis type 3 in the renal medullaJuvenile neuronal ceroid lipofuscinosis (JNCL) and the eye.Exacerbating and reversing lysosomal storage diseases: from yeast to humans.Two motifs target Batten disease protein CLN3 to lysosomes in transfected nonneuronal and neuronal cells.A central role for TOR signalling in a yeast model for juvenile CLN3 diseaseS. pombe btn1, the orthologue of the Batten disease gene CLN3, is required for vacuole protein sorting of Cpy1p and Golgi exit of Vps10p.A novel role of the Batten disease gene CLN3: association with BMP synthesis.Maturation of human tripeptidyl-peptidase I in vitro.A dileucine motif and a cluster of acidic amino acids in the second cytoplasmic domain of the batten disease-related CLN3 protein are required for efficient lysosomal targeting.Glycosaminoglycans modulate activation, activity, and stability of tripeptidyl-peptidase I in vitro and in vivo.Lack of specificity of antibodies raised against CLN3, the lysosomal/endosomal transmembrane protein mutated in juvenile Batten disease.Defective synaptic transmission causes disease signs in a mouse model of juvenile neuronal ceroid lipofuscinosis.Role of the Lysosomal Membrane Protein, CLN3, in the Regulation of Cathepsin D Activity.Altered Expression of Ganglioside Metabolizing Enzymes Results in GM3 Ganglioside Accumulation in Cerebellar Cells of a Mouse Model of Juvenile Neuronal Ceroid Lipofuscinosis.Pathogenic LRRK2 mutations, through increased kinase activity, produce enlarged lysosomes with reduced degradative capacity and increase ATP13A2 expression.C-terminal prenylation of the CLN3 membrane glycoprotein is required for efficient endosomal sorting to lysosomes.H-Rubies, a new family of red emitting fluorescent pH sensors for living cells.Modulating membrane fluidity corrects Batten disease phenotypes in vitro and in vivo.Responsive Contrast Agents: Synthesis and Characterization of a Tunable Series of pH-Sensitive Near-Infrared Pentamethines.Linkage analysis of multiplex Caribbean Hispanic families loaded for unexplained early-onset cases identifies novel Alzheimer's disease lociImmunomodulation with minocycline rescues retinal degeneration in juvenile neuronal ceroid lipofuscinosis mice highly susceptible to light damage
P2860
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P2860
CLN3 protein regulates lysosomal pH and alters intracellular processing of Alzheimer's amyloid-beta protein precursor and cathepsin D in human cells
description
2000 nî lūn-bûn
@nan
2000 թուականի Յուլիսին հրատարակուած գիտական յօդուած
@hyw
2000 թվականի հուլիսին հրատարակված գիտական հոդված
@hy
2000年の論文
@ja
2000年論文
@yue
2000年論文
@zh-hant
2000年論文
@zh-hk
2000年論文
@zh-mo
2000年論文
@zh-tw
2000年论文
@wuu
name
CLN3 protein regulates lysosom ...... and cathepsin D in human cells
@ast
CLN3 protein regulates lysosom ...... and cathepsin D in human cells
@en
CLN3 protein regulates lysosom ...... and cathepsin D in human cells
@en-gb
CLN3 protein regulates lysosom ...... and cathepsin D in human cells
@nl
type
label
CLN3 protein regulates lysosom ...... and cathepsin D in human cells
@ast
CLN3 protein regulates lysosom ...... and cathepsin D in human cells
@en
CLN3 protein regulates lysosom ...... and cathepsin D in human cells
@en-gb
CLN3 protein regulates lysosom ...... and cathepsin D in human cells
@nl
prefLabel
CLN3 protein regulates lysosom ...... and cathepsin D in human cells
@ast
CLN3 protein regulates lysosom ...... and cathepsin D in human cells
@en
CLN3 protein regulates lysosom ...... and cathepsin D in human cells
@en-gb
CLN3 protein regulates lysosom ...... and cathepsin D in human cells
@nl
P2093
P921
P356
P1476
CLN3 protein regulates lysosom ...... and cathepsin D in human cells
@en
P2093
A A Golabek
K E Wisniewski
M Michalewski
W Kaczmarski
P304
P356
10.1006/MGME.2000.3006
P407
P577
2000-07-01T00:00:00Z