The juvenile Batten disease protein, CLN3, and its role in regulating anterograde and retrograde post-Golgi trafficking.
about
Nonmuscle myosin-2: mix and matchLoss of Cln3 function in the social amoeba Dictyostelium discoideum causes pleiotropic effects that are rescued by human CLN3CLN3 deficient cells display defects in the ARF1-Cdc42 pathway and actin-dependent eventsLarge-scale phenotyping of an accurate genetic mouse model of JNCL identifies novel early pathology outside the central nervous systemmTORC1-independent TFEB activation via Akt inhibition promotes cellular clearance in neurodegenerative storage diseasesLife without double-headed non-muscle myosin II motor proteins.Unbiased Cell-based Screening in a Neuronal Cell Model of Batten Disease Highlights an Interaction between Ca2+ Homeostasis, Autophagy, and CLN3 Protein Function.Neuroinflammatory paradigms in lysosomal storage diseases.Comparative deletion mapping at 1p31.3-p32.2 implies NFIA responsible for intellectual disability coupled with macrocephaly and the presence of several other genes for syndromic intellectual disability.Antigen presenting cell abnormalities in the Cln3(-/-) mouse model of juvenile neuronal ceroid lipofuscinosisCLN3 loss disturbs membrane microdomain properties and protein transport in brain endothelial cells.Human iPSC models of neuronal ceroid lipofuscinosis capture distinct effects of TPP1 and CLN3 mutations on the endocytic pathwayCongenic fine-mapping identifies a major causal locus for variation in the native collateral circulation and ischemic injury in brain and lower extremity.Ataluren as an agent for therapeutic nonsense suppression.Detailed Clinical Phenotype and Molecular Genetic Findings in CLN3-Associated Isolated Retinal Degeneration.Aberrant adhesion impacts early development in a Dictyostelium model for juvenile neuronal ceroid lipofuscinosis.Role of the Lysosomal Membrane Protein, CLN3, in the Regulation of Cathepsin D Activity.Revisiting the neuronal localization and trafficking of CLN3 in juvenile neuronal ceroid lipofuscinosis.Altered Expression of Ganglioside Metabolizing Enzymes Results in GM3 Ganglioside Accumulation in Cerebellar Cells of a Mouse Model of Juvenile Neuronal Ceroid Lipofuscinosis.Phenotyping heterozygous carriers of juvenile neuronal ceroid lipofuscinosis with CLN3 mutations.Modulating membrane fluidity corrects Batten disease phenotypes in vitro and in vivo.
P2860
Q27022727-F0A1091C-B845-4E8B-8F08-DD7C18AFA585Q27334931-C810E62E-221E-4A74-82C3-446027C81C64Q28513970-FC8630FC-117E-4947-8D25-53A0FEC58C47Q28728782-01DA68FA-8CEE-4CFF-94EF-C87D76B0FBC7Q30838293-4C17ACEA-D246-44B7-8415-2A97B314EC8BQ33852054-841F0333-67C4-4919-8A18-7E2505859377Q35860759-C81152AE-9492-451B-BAEA-A1CB020FE10AQ36232187-3DC155EF-AF09-43BC-A2F6-43ECE8212C4CQ36700817-B4206D95-3663-443E-84E4-A2A4D74E1D22Q36986268-9364A52F-9E2F-4225-A2FE-9B7E1A716363Q37309514-C4EA9FB4-12E4-4112-919B-7A128E08C0EFQ37649077-4A5267B5-0CBA-4F05-80AD-F5D3096F68DBQ37663600-2F4E16DB-A71F-4A8A-AAB4-2FE2EBFDA557Q37670599-C822F800-9699-4D5A-AD63-7012511156CDQ39329067-E90C7EAE-D623-4CDE-90DF-F5C33FED5D7CQ41510250-8A931F57-AEFB-496B-8278-A2D233249190Q48301421-1F640244-3071-4A5C-B02C-312B640F91A4Q48465441-AE06FC08-474C-4286-810C-285B166A8AB8Q51748958-EB9AD02B-5B42-4004-AED0-F542E084C11EQ52872948-E7D85AB5-847F-4F5D-89A2-1C8BAAB13D53Q55541817-EB8C0D99-73BE-4906-88E4-23D22A7936A0
P2860
The juvenile Batten disease protein, CLN3, and its role in regulating anterograde and retrograde post-Golgi trafficking.
description
2012 nî lūn-bûn
@nan
2012 թուականի Փետրուարին հրատարակուած գիտական յօդուած
@hyw
2012 թվականի փետրվարին հրատարակված գիտական հոդված
@hy
2012年の論文
@ja
2012年論文
@yue
2012年論文
@zh-hant
2012年論文
@zh-hk
2012年論文
@zh-mo
2012年論文
@zh-tw
2012年论文
@wuu
name
The juvenile Batten disease pr ...... ograde post-Golgi trafficking.
@ast
The juvenile Batten disease pr ...... ograde post-Golgi trafficking.
@en
The juvenile Batten disease pr ...... ograde post-Golgi trafficking.
@nl
type
label
The juvenile Batten disease pr ...... ograde post-Golgi trafficking.
@ast
The juvenile Batten disease pr ...... ograde post-Golgi trafficking.
@en
The juvenile Batten disease pr ...... ograde post-Golgi trafficking.
@nl
prefLabel
The juvenile Batten disease pr ...... ograde post-Golgi trafficking.
@ast
The juvenile Batten disease pr ...... ograde post-Golgi trafficking.
@en
The juvenile Batten disease pr ...... ograde post-Golgi trafficking.
@nl
P2860
P356
P1433
P1476
The juvenile Batten disease pr ...... ograde post-Golgi trafficking.
@en
P2093
John F Staropoli
Susan L Cotman
P2860
P356
10.2217/CLP.11.70
P577
2012-02-01T00:00:00Z