Keywords: AQP4; aquaporin-4; CNS; central nervous system; CSF; cerebrospinal fluid; EDSS; Expanded Disability Status Scale; ELISA; enzyme-linked immunosorbent assay; FTLD; frontotemporal lobar degeneration; IL; interleukin; mPSL; methylprednisolone; MRI; magnetic r
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Keywords: BBB; blood-brain barrier; CBD; cortical basal degeneration; CTE; chronic traumatic encephalopathy; EpoD; epothilone D; FTLD; frontotemporal lobar degeneration; MAP; microtubule-associated protein; MT; microtubules; MTOC; microtubule organizing center; PSP
Keywords: AD; Alzheimer's disease; PD; Parkinson's disease; DLB; dementia with Lewy bodies; MSA; multiple system atrophy; ALS; amyotrophic lateral sclerosis; αSyn; α-synuclein; TDP-43; TAR DNA binding protein of 43 kDa; FTLD; frontotemporal lobar degeneration; P
Keywords: Cre; Cre-recombinase; DAVID; Database for Annotation, Visualization and Integrated Discovery; EPM; elevated plus maze; DRL; delayed response learning; FTLD; frontotemporal lobar degeneration; GO; gene ontology; Grn; gene name for progranulin; NOR; novel o
Keywords: Aβ; amyloid-beta; AD; Alzheimer's disease; ALS; amyotrophic lateral sclerosis; APCI; atmospheric pressure chemical ionization; APOE; apolipoprotein E; APP; amyloid precursor protein; APPI; atmospheric pressure photoionization; CE; capillary electrophor
Keywords: AED; antiepileptic drug; EF; executive function; FTLD; frontotemporal lobar degeneration; HC; healthy controls; IGE; idiopathic generalized epilepsy; JME; juvenile myoclonic epilepsy; PFF; paradoxical functional facilitation; SD; standard deviation; TLE;
Keywords: Lexical access; Visual lexical decision; Language; Atrophy; fMRI; Compensation; PPA; FTLD; Dementia; Progression
Keywords: FTLD; Progranulin; Magnetic resonance imaging; Cortical thickness; Diffusion tensor; Resting-state functional MRI;
Keywords: Behavioral variant frontotemporal dementia; Cognitive neuropsychiatry; FDG-PET; Meta-analysis; MRI; AcLE; activation likelihood estimate; AnLE; anatomical likelihood estimate; bvFTD; behavioral variant frontotemporal dementia; DSM; Diagnostic and Statisti
Keywords: Amyotrophic lateral sclerosis; ALS; FTLD; Profilin 1; Cytoskeleton; Actin;
Keywords: TREM2; Alzheimer's disease; FTLD; neurodegeneration;
Keywords: ALS; FET family; FTLD; FUS; Neurodegeneration; Nuclear import; Stress granules; TLS; Transportin;
Keywords: AD; Alzheimer's disease; ALS; amyotrophic lateral sclerosis; EDTA; ethylenediaminetetraacetic acid; FTLD; frontotemporal lobar degeneration; GFAP; glial fibrillary acidic protein; HE; hematoxylin and eosin; HPRT; hypoxanthine phosphoribosyltransferase;
Keywords: AD; Alzheimer's disease; ALS; amyotrophic lateral sclerosis; ANOVA; Analysis of variance; Aβ; amyloid β; bHLH; basic Helix-Loop-Helix; C4; complement C4; CLEAR; coordinated lysosomal expression and regulation; Cyba; cytochrome b-245 light chain; DW; d
Latent classes of mild cognitive impairment are associated with clinical outcomes and neuropathology: Analysis of data from the National Alzheimer's Coordinating Center
Keywords: Aβ; amyloid-beta; AD; Alzheimer's disease; ADC; Alzheimer's Disease Center; AMN; amnestic; CSF; cerebrospinal fluid; DLB; dementia with Lewy bodies; FTLD; frontotemporal lobar degeneration; FX; functional impairments; GEE; generalized estimating equation
Muscle-dominant wild-type TDP-43 expression induces myopathological changes featuring tubular aggregates and TDP-43-positive inclusions
Keywords: Sporadic inclusion body myositis; TAR DNA-binding protein of 43â¯kDa; Creatine kinase 8 promoter; Endoplasmic reticulum stress; Calcium homeostasis; AST; aspartate aminotransferase; Aβ; amyloid β; ALS; amyotrophic lateral sclerosis; BiP; immunoglobulin
FTLD/ALS-linked TDP-43 mutations do not alter TDP-43's ability to self-regulate its expression in Drosophila
Keywords: TDP-43; FTLD; ALS; Drosophila; Autoregulation; Mutation;
The physiological and pathological biophysics of phase separation and gelation of RNA binding proteins in amyotrophic lateral sclerosis and fronto-temporal lobar degeneration
Keywords: ADMA FUS; asymmetrically di-methylated arginine FUS; CHOP; C/EBP homologous protein gene; DDX4; DEAD box helicase 4; EWS; Ewing sarcoma protein; fALS; familial amyotrophic lateral sclerosis; FTLD; frontotemporal lobar degeneration; FUS; fused in sarcoma p
α-Methyl-α-phenylsuccinimide ameliorates neurodegeneration in a C. elegans model of TDP-43 proteinopathy
Keywords: MPS; α-methyl-α-phenylsuccinimide; AD; Alzheimer's disease; ALS; Amyotrophic lateral sclerosis; BSA; Bovine serum albumin; CNS; Central nervous system; DRS; Dent's Ringer solution; FTLD; Frontotemporal lobar degeneration; MOO; Multi-objective optimisati
TAR DNA-Binding Protein 43 and Disrupted in Schizophrenia 1 Coaggregation Disrupts Dendritic Local Translation and Mental Function in Frontotemporal Lobar Degeneration
Keywords: Coaggregation; DISC1; FTLD; Local translation; Psychiatric behaviors; TDP-43;
Oligodendrogliopathy in neurodegenerative diseases with abnormal protein aggregates: The forgotten partner
Keywords: 5-HT; 5-hydroxytryptamine; Aβ; β-amyloid; ABCA8; ATP binding cassette subfamily A member 8; AD; Alzheimer's disease; ADP; adenosine diphosphate; AGD; argyrophilic grain disease; ALS; amyotrophic lateral sclerosis; AMBRA-1; autophagy and beclin 1 regul
Oxidative stress and mitochondrial damage in the pathogenesis of ALS: New perspectives
Keywords: ALS; Amyotrophic Lateral Sclerosis; FTLD; Frontotemporal Lobar Degeneration; mPOS; mitochondrial precursor over-accumulation stress; ROS; Reactive Oxygen Species; UPRam; Unfolded Protein Response Activated by Mistargeting of proteins; UPRmt; mitochondrial
Evaluation of early-phase [18F]-florbetaben PET acquisition in clinical routine cases
Keywords: PET; Positron emission tomography; FBB; [18F]florbetaben; FDG; [18F]-fluorodeoxyglucose; 3D-SSP; 3-dimensional stereotactic surface projections; p.i.; post injection; AD; Alzheimer's disease; SPECT; single photon emission computed tomography; CBF; cerebra
TDP-43 proteolysis is associated with astrocyte reactivity after traumatic brain injury in rodents
Keywords: ALS; Amyotrophic lateral sclerosis; ANOVA; A two-way analysis of variance; FTLD; Frontotemporal lobar degeneration; GFAP; Glial fibrillary acidic protein; TBI; Traumatic brain injury; TDP-43; Transactivation response DNA-binding protein 43; TX; High-salt
Enhancing Mitofusin/Marf ameliorates neuromuscular dysfunction in Drosophila models of TDP-43 proteinopathies
Keywords: ALS; FTLD; Mitochondrial dynamics; Mitochondria; Dynamin-related protein 1;
The relevance of contact-independent cell-to-cell transfer of TDP-43 and SOD1 in amyotrophic lateral sclerosis
Keywords: ALS; amyotrophic lateral sclerosis; CFTR; cystic fibrosis transmembrane conductance regulator; CNS; central nervous system; CSF; cerebrospinal fluid; fALS; familial ALS; FTLD; frontotemporal lobar degeneration; LMN; lower motor neurons; sALS; sporadic ALS
Genetic analysis of TREM2 variants in Chinese Han patients with sporadic Parkinson's disease
Keywords: PD; Parkinson's disease; AD; Alzheimer's disease; TREM2; the triggering receptor expressed on myeloid cells 2 gene; VPS35; the vacuolar protein sorting 35 gene; EIF4G1; the eukaryotic translation initiation factor 4-gamma 1 gene; S100B; the S100 calci
ALS-FTLD associated mutations of SQSTM1 impact on Keap1-Nrf2 signalling
Keywords: ALS; Amyotrophic lateral sclerosis; ARE; Antioxidant response element; FTLD; Frontotemporal lobar degeneration; IKKβ; Inhibitor of nuclear factor kappa-B kinase subunit beta; Keap1; Kelch-like ECH-associated protein 1; KIR; Keap1-interacting region; LIR;
TDP-43/FUS in motor neuron disease: Complexity and challenges
Keywords: AD; Alzheimer's disease; Ago2; Argonaute 2; ALS; Amyotrophic lateral sclerosis; ALS/PD; amyotrophic lateral sclerosis and Parkinsonism-dementia complex; AMPA; α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid; ATF3; activating transcription factor 3
A new simple score (ABS) for assessing behavioral and psychological symptoms of dementia
Keywords: ABS; Abe's BPSD score; AD; Alzheimer's disease; ADAS-Cog; Alzheimer's Disease Assessment Scale-Cognitive Section; Behave-AD; behavioral pathology in AD; BPSD; behavioral and psychological symptoms of dementia; CDR; Clinical Dementia Rating Scale; CGBRS;
Bridging NCL research gaps
Keywords: NCL; neuronal ceroid lipofuscinosis; LSD; lysosomal storage disease; ERT; enzyme replacement therapy; ER; endoplasmic reticulum; PoC; proof-of-concept; iPS cell; induced pluripotent stem cell; PD; Parkinson's disease; NPC; Niemann-Pick type C; SLOS; Smi
p62/SQSTM1 analysis in frontotemporal lobar degeneration
Keywords: p62; SQSTM1; C9orf72; Frontotemporal lobar degeneration; FTLD;
A small deletion in C9orf72 hides a proportion of expansion carriers in FTLD
Keywords: Frontotemporal lobar degeneration; FTLD; C9orf72; Repeat expansion; ALS;
TDP-35 sequesters TDP-43 into cytoplasmic inclusions through binding with RNA
Keywords: ALS; amyotrophic lateral sclerosis; DNase; deoxyribonuclease; FISH; fluorescent in situ hybridization; FTLD; frontotemporal lobar degeneration; GFP; green fluorescent protein; IF; immunofluorescence; ITC; isothermal titration calorimetry; RIP; RNA immunop
Autophagy receptor defects and ALS-FTLD
Keywords: ALS; FTLD; SQSTM1/p62; Optineurin; VCP; Ubiquilin; Autophagy; Stress granules;
Traumatic brain injury causes frontotemporal dementia and TDP-43 proteolysis
Keywords: ALS; amyotrophic lateral sclerosis; CI; confidence interval; FTD; frontotemporal dementia; FTLD; frontotemporal lobar degeneration; HRs; hazard ratios; SARK; sarkosyl; TBI; traumatic brain injury; TDP-43; TAR-DNA binding protein 43; traumatic brain injury
Overexpression of nuclear FUS induces neuronal cell death
Keywords: ALS; amyotrophic lateral sclerosis; DMEM; Dulbecco's modified Eagle's medium; FBS; fetal bovine serum; FITC; fluorescein isothiocyanate; FTLD; frontotemporal lobar degeneration; FUS; fused in sarcoma; GAPDH; glyceraldehyde-3-phosphate dehydrogenase; L
Nuclear import factor transportin and arginine methyltransferase 1 modify FUS neurotoxicity in Drosophila
Keywords: FUS; Fused in Sarcoma; ALS; amyotrophic lateral sclerosis; FTLD; frontotemporal lobar degeneration; VNC; ventral nerve cord; NLS; nuclear localization signal; Caz; Cabeza; PRMT; protein arginine methyltransferase; DART; Drosophila arginine methyltransfera
Frontotemporal lobar dementia and amyotrophic lateral sclerosis associated with c9orf72 expansion
Keywords: Frontotemporal dementia; FTLD; FTLD-ALS; ALS; PGRN; C9ORF72; MAPT; TDP-43; Loss-of-function; RNA foci; Dipeptide repeats; Démence lobaire frontotemporale; DLFT; Sclérose latérale amyotrophique; SLA; DLFT-SLA; PGRN; C9ORF72; MAPT; TDP-43; Perte de fonct
Different mutations at V363 MAPT codon are associated with atypical clinical phenotypes and show unusual structural and functional features
Keywords: Frontotemporal lobar degeneration; FTLD; PNFA; PSP; PCA; MAPT gene; Mutation; Pathogenicity; Tau; Microtubules; Oligomers; Fibrils;
Reduced cellular Ca2+ availability enhances TDP-43 cleavage by apoptotic caspases
Keywords: ALS; amyotrophic lateral sclerosis; BAPTA-AM; 1,2-bis (o-aminophenoxy) ethane-N,N,Nâ²,Nâ²-tetraacetic acid acetoxymethyl ester; BSA; bovine serum albumin; CHAPS; 3-[(3-cholamidopropyl) dimethylammonio]-1-propanesulfonate; CoCl2; cobalt chloride; DMEM; D
The ALS/FTLD-related RNA-binding proteins TDP-43 and FUS have common downstream RNA targets in cortical neurons
Keywords: ALS; FTLD; TDP-43; FUS; TranscriptomeALS, amyotrophic lateral sclerosis; Cugbp1, CUG triplet repeat, RNA-binding protein 1; DAVID, Database for Annotation, Visualization and Integrated Discovery; FTLD, frontotemporal lobar degeneration; FUS, fused in sa
SQSTM1 mutations - Bridging Paget disease of bone and ALS/FTLD
Keywords: ALS; amyotrophic lateral sclerosis; aPKC; atypical protein kinase C; C9orf72; chromosome 9 open reading frame 72; FTD; frontotemporal dementia; FTLD; frontotemporal lobar degeneration; FUS/TLS; fused in sarcoma/translocated in liposarcoma; GWAS; genome-wi
Methyl pyruvate rescues mitochondrial damage caused by SIGMAR1 mutation related to amyotrophic lateral sclerosis
Keywords: ALS; amyotrophic lateral sclerosis; DMEM; Dulbecco's minimal essential medium; ER; endoplasmic reticulum; FBS; fetal bovine serum; FTLD; frontotemporal lobar degeneration; GRP75; 75Â kDa glucose-regulated protein; IP3; inositol 1,4,5-trisphosphate; IP3R;
Explorative genetic study of UBQLN2 and PFN1 in an extended Flanders-Belgian cohort of frontotemporal lobar degeneration patients
Keywords: UBQLN2; PFN1; FTLD; ALS;
Molecular mechanism of oxidation-induced TDP-43 RRM1 aggregation and loss of function
Keywords: TDP-43; trans-activation region DNA-binding protein of 43-kDa; RRM; RNA recognition motif; ALS; amyotrophic lateral sclerosis; FTLD; frontotemporal lobar degeneration; AD; Alzheimer's disease; PD; Parkinson's disease; HD; Huntington's disease; NTD;
Progranulin directly binds to the CRD2 and CRD3 of TNFR extracellular domains
Keywords: PGRN; progranulin; TNFR; tumor-necrotizing factor receptor; CRD; cysteine-rich domains; BMDM; bone marrow-derived macrophages; SPR; Surface plasmon resonance; FTLD; frontotemporal lobar degeneration; Y2H; yeast two-hybrid; HRP; horseradish peroxidase; PGR
A cellular model for sporadic ALS using patient-derived induced pluripotent stem cells
Keywords: ALS; amyotrophic lateral sclerosis; iPSC; induced pluripotent stem cells; TDP-43; TARDBP, TAR DNA binding protein; SOD1; superoxide dismutase 1; FTLD; fronto-temporal lobar degeneration; iPSC-MN; induced pluripotent stem cell-derived motor neurons; iPSC-C
Epitope mapping of antibodies against TDP-43 and detection of protease-resistant fragments of pathological TDP-43 in amyotrophic lateral sclerosis and frontotemporal lobar degeneration
Keywords: Aggregation; Tau; Alpha-synuclein; ALS; FTLD
Recent advances in p97/VCP/Cdc48 cellular functions
Keywords: AAA; ATPases associated with diverse cellular activities; VCP; valosin-containing protein; ER; endoplasmic reticulum; ERAD; ER associated degradation; IBMPFD; inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia; ALS;