CLN3 protein is targeted to neuronal synapses but excluded from synaptic vesicles: new clues to Batten disease
about
Exome-sequencing confirms DNAJC5 mutations as cause of adult neuronal ceroid-lipofuscinosisRetinitis pigmentosaA 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 compartmentsMembrane trafficking and mitochondrial abnormalities precede subunit c deposition in a cerebellar cell model of juvenile neuronal ceroid lipofuscinosisThe neuronal ceroid lipofuscinosis Cln8 gene expression is developmentally regulated in mouse brain and up-regulated in the hippocampal kindling model of epilepsy.Loss of Cln3 function in the social amoeba Dictyostelium discoideum causes pleiotropic effects that are rescued by human CLN3Increased expression of lysosomal acid phosphatase in CLN3-defective cells and mouse brain tissueNovel interactions of CLN5 support molecular networking between Neuronal Ceroid Lipofuscinosis proteinsCLN3p impacts galactosylceramide transport, raft morphology, and lipid contentInteractions of the proteins of neuronal ceroid lipofuscinosis: clues to functionCLN3 deficient cells display defects in the ARF1-Cdc42 pathway and actin-dependent eventsExpression and lysosomal targeting of CLN7, a major facilitator superfamily transporter associated with variant late-infantile neuronal ceroid lipofuscinosisLarge-scale phenotyping of an accurate genetic mouse model of JNCL identifies novel early pathology outside the central nervous systemData 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.Phosphatidylinositol-4-kinase type II alpha is a component of adaptor protein-3-derived vesicles.A metabolomic comparison of mouse models of the Neuronal Ceroid Lipofuscinoses.The juvenile Batten disease protein, CLN3, and its role in regulating anterograde and retrograde post-Golgi trafficking.The genetic spectrum of human neuronal ceroid-lipofuscinoses.Osmotic stress changes the expression and subcellular localization of the Batten disease protein CLN3.The neuronal ceroid-lipofuscinoses.Selectivity and types of cell death in the neuronal ceroid lipofuscinoses.Photoreceptor phagosome processing defects and disturbed autophagy in retinal pigment epithelium of Cln3Δex1-6 mice modelling juvenile neuronal ceroid lipofuscinosis (Batten disease).Neuronal ceroid lipofuscinosis with DNAJC5/CSPα mutation has PPT1 pathology and exhibit aberrant protein palmitoylation.Synaptic pathology in retinoschisis knockout (Rs1-/y) mouse retina and modification by rAAV-Rs1 gene deliveryThe zinc-binding domain of Nna1 is required to prevent retinal photoreceptor loss and cerebellar ataxia in Purkinje cell degeneration (pcd) mice.Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease.Neuronal ceroid lipofuscinosis: impact of recent genetic advances and expansion of the clinicopathologic spectrum.btn1 affects endocytosis, polarization of sterol-rich membrane domains and polarized growth in Schizosaccharomyces pombe.AP-1 and AP-3 facilitate lysosomal targeting of Batten disease protein CLN3 via its dileucine motif.Two motifs target Batten disease protein CLN3 to lysosomes in transfected nonneuronal and neuronal cells.Proteomic mapping of differentially vulnerable pre-synaptic populations identifies regulators of neuronal stability in vivoin vivo localization of the neuronal ceroid lipofuscinosis proteins, CLN3 and CLN7, at endogenous expression levelsS. pombe btn1, the orthologue of the Batten disease gene CLN3, is required for vacuole protein sorting of Cpy1p and Golgi exit of Vps10p.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.The Niemann-Pick C1 protein in recycling endosomes of presynaptic nerve terminals.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.Revisiting the neuronal localization and trafficking of CLN3 in juvenile neuronal ceroid lipofuscinosis.
P2860
Q21090977-3D8FC77E-FAD4-47E5-8ABF-021F19509BC2Q21203028-85416F6E-691D-4D7D-A9A9-1D45E7E602C0Q24298716-4F8C8D23-2A89-4B04-9A42-463D6A23E3EBQ24301862-27C002F9-840C-450C-87D6-80EF3E2AB3F3Q24302244-DA7FADD3-DE7E-43F1-B459-8A171D1E01DBQ24791822-2598CC26-B2A0-4722-ABC9-ACD6CC525B3EQ24793088-854C0147-014E-4FEB-AB03-69DAE4455C95Q27334931-6E62ED00-1E63-4BB7-B31E-BF06E590114FQ28114924-8C4C382B-9E78-4893-897C-E9982EFDD2B5Q28115540-661316FE-C366-4426-A9D0-9E5D889136A1Q28271390-7C0F07AD-A205-492B-B02B-293372C00113Q28289653-FC9D97E8-669A-4382-AEFF-0A929435932AQ28513970-93FA832A-CE50-4675-975C-6C8E3919FD13Q28589863-825EB333-71CC-46C3-88BE-EFBEA44F1F6CQ28728782-D4E36594-3CD7-45AF-81C0-63013A9E1DB4Q31120806-1730ECA0-5B45-4787-9844-A26BF39DD47FQ33911122-BA741DE1-D32A-4107-AB99-C6DDA3676E5BQ34007930-D11E0294-6F22-47E9-A42C-082F1B922607Q34271805-E4EB822E-D29C-427B-A9AA-6855E435471FQ34302797-7226652C-4A8B-4EF2-AA63-9CA99FD3D864Q34805162-A146715C-CDB6-4E83-8F40-F9B535A966EEQ35045618-07F1990C-18A5-41A8-9789-B33E83524D4AQ35681068-F9D58E4F-E9CA-4E7D-97FC-9753C30110F9Q36302113-EEC571A6-BB42-44B1-AFAE-D9BE363522EFQ36685260-4DD8C3EA-A120-4221-819D-62A2558357EAQ36915061-D61621F6-E8BC-449D-9AE1-B8622B14139FQ37014856-82F814FF-3E3C-441D-8036-D176A0309D9AQ37378095-62B8E4E9-C8CC-4B3D-9DC8-2E9B58D93F0BQ38115191-C8B106F7-BDCE-4906-BD1B-27E8DE0B4F67Q39598497-F96516D8-14F0-41AC-BB06-82BD503ABC4EQ40480798-9EA861A4-7149-499B-96E9-4282D46C673AQ40648630-7C32C778-0DA4-466A-8E69-FD227B47815DQ42069362-4F004648-02DE-4965-B387-F046989FC515Q42250217-3DC78C46-D1D7-4972-A67F-658323F49F35Q42563162-956166B1-09E3-4D0C-98B6-B512FE543402Q45094647-C8B41EA6-9297-484A-9571-DDF265C13767Q46844440-12575BA7-C541-4180-9D79-727EA25ADBDFQ47134547-46805F63-ECB4-421E-BC27-981EFA4FE7DEQ47140069-658253F3-D549-4D0A-995F-51132159B0A2Q48465441-AC095BA9-B792-4C60-A656-33CF24213A1C
P2860
CLN3 protein is targeted to neuronal synapses but excluded from synaptic vesicles: new clues to Batten disease
description
2001 nî lūn-bûn
@nan
2001 թուականի Սեպտեմբերին հրատարակուած գիտական յօդուած
@hyw
2001 թվականի սեպտեմբերին հրատարակված գիտական հոդված
@hy
2001年の論文
@ja
2001年学术文章
@wuu
2001年学术文章
@zh-cn
2001年学术文章
@zh-hans
2001年学术文章
@zh-my
2001年学术文章
@zh-sg
2001年學術文章
@yue
name
CLN3 protein is targeted to ne ...... s: new clues to Batten disease
@ast
CLN3 protein is targeted to ne ...... s: new clues to Batten disease
@en
CLN3 protein is targeted to ne ...... s: new clues to Batten disease
@en-gb
CLN3 protein is targeted to ne ...... s: new clues to Batten disease
@nl
type
label
CLN3 protein is targeted to ne ...... s: new clues to Batten disease
@ast
CLN3 protein is targeted to ne ...... s: new clues to Batten disease
@en
CLN3 protein is targeted to ne ...... s: new clues to Batten disease
@en-gb
CLN3 protein is targeted to ne ...... s: new clues to Batten disease
@nl
prefLabel
CLN3 protein is targeted to ne ...... s: new clues to Batten disease
@ast
CLN3 protein is targeted to ne ...... s: new clues to Batten disease
@en
CLN3 protein is targeted to ne ...... s: new clues to Batten disease
@en-gb
CLN3 protein is targeted to ne ...... s: new clues to Batten disease
@nl
P2093
P3181
P356
P1476
CLN3 protein is targeted to ne ...... s: new clues to Batten disease
@en
P2093
P304
P3181
P356
10.1093/HMG/10.19.2123
P407
P50
P577
2001-09-01T00:00:00Z