Action of BTN1, the yeast orthologue of the gene mutated in Batten disease.
about
Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)Defective lysosomal arginine transport in juvenile Batten diseaseA galactosylceramide binding domain is involved in trafficking of CLN3 from Golgi to rafts via recycling endosomesNeuronal ceroid lipofuscinoses are connected at molecular level: interaction of CLN5 protein with CLN2 and CLN3Neuronal ceroid lipofuscinosis protein CLN3 interacts with motor proteins and modifies location of late endosomal compartmentsClarifying lysosomal storage diseasesMembrane trafficking and mitochondrial abnormalities precede subunit c deposition in a cerebellar cell model of juvenile neuronal ceroid lipofuscinosisYeast prions: structure, biology, and prion-handling systemsDistinct early molecular responses to mutations causing vLINCL and JNCL presage ATP synthase subunit C accumulation in cerebellar cellsCuring of the [URE3] prion by Btn2p, a Batten disease-related protein.Btn2, a Hook1 ortholog and potential Batten disease-related protein, mediates late endosome-Golgi protein sorting in yeast.A role in vacuolar arginine transport for yeast Btn1p and for human CLN3, the protein defective in Batten disease.Saccharomyces cerevisiae lacking Btn1p modulate vacuolar ATPase activity to regulate pH imbalance in the vacuole.Interaction among Btn1p, Btn2p, and Ist2p reveals potential interplay among the vacuole, amino acid levels, and ion homeostasis in the yeast Saccharomyces cerevisiaeInteraction with Btn2p is required for localization of Rsglp: Btn2p-mediated changes in arginine uptake in Saccharomyces cerevisiae.Btn3 is a negative regulator of Btn2-mediated endosomal protein trafficking and prion curing in yeast.The yeast model for batten disease: mutations in BTN1, BTN2, and HSP30 alter pH homeostasis.Increased expression of lysosomal acid phosphatase in CLN3-defective cells and mouse brain tissueVaried mechanisms underlie the free sialic acid storage disordersAutophagy is disrupted in a knock-in mouse model of juvenile neuronal ceroid lipofuscinosisAlterations in ROS activity and lysosomal pH account for distinct patterns of macroautophagy in LINCL and JNCL fibroblasts.Interaction between Sdo1p and Btn1p in the Saccharomyces cerevisiae model for Batten disease.Normal levels of the antiprion proteins Btn2 and Cur1 cure most newly formed [URE3] prion variantsAltered transcription in yeast expressing expanded polyglutamine.A metabolomic comparison of mouse models of the Neuronal Ceroid Lipofuscinoses.A novel interaction of CLN3 with nonmuscle myosin-IIB and defects in cell motility of Cln3(-/-) cellsFlupirtine blocks apoptosis in batten patient lymphoblasts and in human postmitotic CLN3- and CLN2-deficient neurons.The juvenile Batten disease protein, CLN3, and its role in regulating anterograde and retrograde post-Golgi trafficking.Neurodevelopmental delay in the Cln3Deltaex7/8 mouse model for Batten disease.The neuronal ceroid-lipofuscinoses.The transient receptor potential channel on the yeast vacuole is mechanosensitive.The yeast Batten disease orthologue Btn1 controls endosome-Golgi retrograde transport via SNARE assembly.Phenotypic reversal of the btn1 defects in yeast by chloroquine: a yeast model for Batten disease.The intracellular location and function of proteins of neuronal ceroid lipofuscinoses.BTN1, the Saccharomyces cerevisiae homolog to the human Batten disease gene, is involved in phospholipid distribution.The cell biology of lysosomal storage disorders.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.Neuroinflammatory paradigms in lysosomal storage diseases.Osmoregulation of ceroid neuronal lipofuscinosis type 3 in the renal medulla
P2860
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P2860
Action of BTN1, the yeast orthologue of the gene mutated in Batten disease.
description
1999 nî lūn-bûn
@nan
1999 թուականի Մայիսին հրատարակուած գիտական յօդուած
@hyw
1999 թվականի մայիսին հրատարակված գիտական հոդված
@hy
1999年の論文
@ja
1999年論文
@yue
1999年論文
@zh-hant
1999年論文
@zh-hk
1999年論文
@zh-mo
1999年論文
@zh-tw
1999年论文
@wuu
name
Action of BTN1, the yeast orthologue of the gene mutated in Batten disease.
@ast
Action of BTN1, the yeast orthologue of the gene mutated in Batten disease.
@en
Action of BTN1, the yeast orthologue of the gene mutated in Batten disease.
@nl
type
label
Action of BTN1, the yeast orthologue of the gene mutated in Batten disease.
@ast
Action of BTN1, the yeast orthologue of the gene mutated in Batten disease.
@en
Action of BTN1, the yeast orthologue of the gene mutated in Batten disease.
@nl
prefLabel
Action of BTN1, the yeast orthologue of the gene mutated in Batten disease.
@ast
Action of BTN1, the yeast orthologue of the gene mutated in Batten disease.
@en
Action of BTN1, the yeast orthologue of the gene mutated in Batten disease.
@nl
P2093
P356
P1433
P1476
Action of BTN1, the yeast orthologue of the gene mutated in Batten disease.
@en
P2093
P2888
P356
10.1038/8861
P407
P577
1999-05-01T00:00:00Z
P5875
P6179
1053126853