| scholarly article | Q13442814 |
| P356 | DOI | 10.1111/BPA.12486 |
| P698 | PubMed publication ID | 28100023 |
| P2093 | author name string | David M A Mann | |
| Julie S Snowden | |||
| P2860 | cites work | Progranulin in frontotemporal lobar degeneration and neuroinflammation | Q21245242 |
| Mutations in the endosomal ESCRTIII-complex subunit CHMP2B in frontotemporal dementia | Q24309521 | ||
| TDP-43 regulates retinoblastoma protein phosphorylation through the repression of cyclin-dependent kinase 6 expression | Q24318430 | ||
| Classification of primary progressive aphasia and its variants | Q24594803 | ||
| Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia | Q24598142 | ||
| Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia | Q24600027 | ||
| Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS | Q24608159 | ||
| Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease | Q24628685 | ||
| Characterization of frontotemporal dementia and/or amyotrophic lateral sclerosis associated with the GGGGCC repeat expansion in C9ORF72 | Q24630288 | ||
| Neuroimaging signatures of frontotemporal dementia genetics: C9ORF72, tau, progranulin and sporadics | Q24630362 | ||
| A harmonized classification system for FTLD-TDP pathology | Q24630756 | ||
| A seeding reaction recapitulates intracellular formation of Sarkosyl-insoluble transactivation response element (TAR) DNA-binding protein-43 inclusions | Q24632492 | ||
| Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS | Q24633692 | ||
| A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD | Q24634583 | ||
| A new subtype of frontotemporal lobar degeneration with FUS pathology | Q24647697 | ||
| The epidemiology of frontotemporal dementia | Q26823243 | ||
| Survival in Frontotemporal Dementia Phenotypes: A Meta-Analysis | Q28079429 | ||
| Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis | Q28131672 | ||
| Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis | Q28236796 | ||
| Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6 | Q28236805 | ||
| Transportin1: a marker of FTLD-FUS | Q28245695 | ||
| Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21 | Q28253639 | ||
| Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17 | Q28253651 | ||
| TDP-43, the signature protein of FTLD-U, is a neuronal activity-responsive factor | Q28261458 | ||
| FUS pathology in basophilic inclusion body disease | Q28261473 | ||
| TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis | Q28270779 | ||
| Familial non-specific dementia maps to chromosome 3 | Q28272020 | ||
| Association of missense and 5'-splice-site mutations in tau with the inherited dementia FTDP-17 | Q28274687 | ||
| Distinct behavioural profiles in frontotemporal dementia and semantic dementia | Q29011731 | ||
| Stages of pTDP-43 pathology in amyotrophic lateral sclerosis | Q37202139 | ||
| Distinct Subtypes of Behavioral Variant Frontotemporal Dementia Based on Patterns of Network Degeneration | Q37258964 | ||
| Mutations in progranulin (GRN) within the spectrum of clinical and pathological phenotypes of frontotemporal dementia | Q37260852 | ||
| C9orf72 frontotemporal lobar degeneration is characterised by frequent neuronal sense and antisense RNA foci. | Q37316053 | ||
| TDP-43 A315T mutation in familial motor neuron disease | Q37354490 | ||
| Clinicopathological and imaging correlates of progressive aphasia and apraxia of speech | Q37356674 | ||
| RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia | Q37409251 | ||
| The frontotemporal syndrome of ALS is associated with poor survival | Q37416385 | ||
| The heritability and genetics of frontotemporal lobar degeneration. | Q37425094 | ||
| Fine structural analysis of the neuronal inclusions of frontotemporal lobar degeneration with TDP-43 proteinopathy. | Q37461525 | ||
| Dipeptide repeat proteins are present in the p62 positive inclusions in patients with frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72. | Q37485777 | ||
| Sequential distribution of pTDP-43 pathology in behavioral variant frontotemporal dementia (bvFTD). | Q37678074 | ||
| Reduced C9orf72 protein levels in frontal cortex of amyotrophic lateral sclerosis and frontotemporal degeneration brain with the C9ORF72 hexanucleotide repeat expansion | Q37706316 | ||
| Frontotemporal dementia caused by CHMP2B mutations | Q37827517 | ||
| Neuropathological background of phenotypical variability in frontotemporal dementia | Q37879961 | ||
| Motor neuron disease and frontotemporal dementia: sometimes related, sometimes not. | Q38163652 | ||
| Timing and significance of pathological features in C9orf72 expansion-associated frontotemporal dementia | Q38377569 | ||
| Semantic dementia: demography, familial factors and survival in a consecutive series of 100 cases | Q38378521 | ||
| TARDBP mutations in motoneuron disease with frontotemporal lobar degeneration | Q38382616 | ||
| Frequency and clinical characteristics of progranulin mutation carriers in the Manchester frontotemporal lobar degeneration cohort: comparison with patients with MAPT and no known mutations | Q38392876 | ||
| Distinct clinical and pathological phenotypes in frontotemporal dementia associated with MAPT, PGRN and C9orf72 mutations | Q38401876 | ||
| Progranulin gene mutations associated with frontotemporal dementia and progressive non-fluent aphasia | Q38403477 | ||
| Histopathological changes underlying frontotemporal lobar degeneration with clinicopathological correlation | Q38411857 | ||
| Frontotemporal lobar degeneration: demographic characteristics of 353 patients | Q38414577 | ||
| Knowledge of famous faces and names in semantic dementia | Q38423596 | ||
| Left/right asymmetry of atrophy in semantic dementia: behavioral-cognitive implications | Q38425469 | ||
| Inherited frontotemporal dementia in nine British families associated with intronic mutations in the tau gene. | Q38435579 | ||
| Classification and pathology of primary progressive aphasia | Q38442670 | ||
| Tau gene mutation K257T causes a tauopathy similar to Pick's disease | Q38442790 | ||
| Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria | Q29614410 | ||
| Mutations of optineurin in amyotrophic lateral sclerosis | Q29614836 | ||
| TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis | Q29616311 | ||
| Inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia is caused by mutant valosin-containing protein | Q29619232 | ||
| Auditory hedonic phenotypes in dementia: A behavioural and neuroanatomical analysis. | Q30374290 | ||
| Common variants at 7p21 are associated with frontotemporal lobar degeneration with TDP-43 inclusions | Q30434949 | ||
| The spectrum of mutations in progranulin: a collaborative study screening 545 cases of neurodegeneration | Q30495415 | ||
| Genetic and clinical features of progranulin-associated frontotemporal lobar degeneration | Q30503638 | ||
| Primary progressive aphasia: a tale of two syndromes and the rest | Q30514205 | ||
| Disruption of structural connectivity along the dorsal and ventral language pathways in patients with nonfluent and semantic variant primary progressive aphasia: a DT MRI study and a literature review | Q30657574 | ||
| Psychosis and hallucinations in frontotemporal dementia with the C9ORF72 mutation: a detailed clinical cohort | Q30670647 | ||
| Semantic dementia: a unique clinicopathological syndrome | Q33303080 | ||
| TDP-43 expression in mouse models of amyotrophic lateral sclerosis and spinal muscular atrophy | Q33379948 | ||
| Language networks in semantic dementia | Q33569984 | ||
| Frontal lobe dementia and motor neuron disease | Q33588620 | ||
| Sequestration of multiple RNA recognition motif-containing proteins by C9orf72 repeat expansions | Q33784052 | ||
| Frequency of ubiquitin and FUS-positive, TDP-43-negative frontotemporal lobar degeneration | Q33830991 | ||
| FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration | Q33922335 | ||
| Clinical and neuroanatomical signatures of tissue pathology in frontotemporal lobar degeneration | Q34028511 | ||
| Syndromes of nonfluent primary progressive aphasia: a clinical and neurolinguistic analysis | Q34099186 | ||
| Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress | Q34157304 | ||
| FET proteins TAF15 and EWS are selective markers that distinguish FTLD with FUS pathology from amyotrophic lateral sclerosis with FUS mutations | Q34209561 | ||
| Survival in frontotemporal dementia | Q34221554 | ||
| The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS. | Q34326849 | ||
| Caudate atrophy on MRI is a characteristic feature of FTLD-FUS | Q34340214 | ||
| High prevalence of mutations in the microtubule-associated protein tau in a population study of frontotemporal dementia in the Netherlands | Q34388804 | ||
| Brain distribution of dipeptide repeat proteins in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72 | Q34500097 | ||
| Ubiquitinated pathological lesions in frontotemporal lobar degeneration contain the TAR DNA-binding protein, TDP-43. | Q34601207 | ||
| Autosomal dominant frontotemporal lobar degeneration due to the C9ORF72 hexanucleotide repeat expansion: late-onset psychotic clinical presentation | Q34615259 | ||
| Psychotic symptoms in frontotemporal dementia: prevalence and review | Q34737838 | ||
| alpha-internexin is present in the pathological inclusions of neuronal intermediate filament inclusion disease | Q35098434 | ||
| Progranulin (granulin-epithelin precursor, PC-cell-derived growth factor, acrogranin) mediates tissue repair and tumorigenesis | Q35203303 | ||
| Frontotemporal lobar degeneration: defining phenotypic diversity through personalized medicine | Q35204586 | ||
| A comparative clinical, pathological, biochemical and genetic study of fused in sarcoma proteinopathies | Q35206942 | ||
| Autopsy proven sporadic frontotemporal dementia due to microvacuolar-type histology, with onset at 21 years of age | Q35482712 | ||
| Psychotic symptoms in frontotemporal dementia: a diagnostic dilemma? | Q35550855 | ||
| Poly-dipeptides encoded by the C9orf72 repeats bind nucleoli, impede RNA biogenesis, and kill cells | Q35695216 | ||
| Antisense RNA foci in the motor neurons of C9ORF72-ALS patients are associated with TDP-43 proteinopathy. | Q35746397 | ||
| Whole-genome sequencing reveals important role for TBK1 and OPTN mutations in frontotemporal lobar degeneration without motor neuron disease | Q35752114 | ||
| Dipeptide repeat protein inclusions are rare in the spinal cord and almost absent from motor neurons in C9ORF72 mutant amyotrophic lateral sclerosis and are unlikely to cause their degeneration. | Q35776562 | ||
| Clinical and pathological features of familial frontotemporal dementia caused by C9ORF72 mutation on chromosome 9p | Q35776895 | ||
| Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations | Q35776901 | ||
| Clinical and neuropathologic variation in neuronal intermediate filament inclusion disease | Q35927831 | ||
| Strikingly different clinicopathological phenotypes determined by progranulin-mutation dosage | Q36017101 | ||
| Distribution of dipeptide repeat proteins in cellular models and C9orf72 mutation cases suggests link to transcriptional silencing | Q36073675 | ||
| Pain and temperature processing in dementia: a clinical and neuroanatomical analysis | Q36206029 | ||
| Antisense proline-arginine RAN dipeptides linked to C9ORF72-ALS/FTD form toxic nuclear aggregates that initiate in vitro and in vivo neuronal death | Q36246294 | ||
| Nuclear carrier and RNA-binding proteins in frontotemporal lobar degeneration associated with fused in sarcoma (FUS) pathological changes. | Q36342066 | ||
| C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD | Q36353773 | ||
| Neurodegeneration. C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits | Q36407722 | ||
| Mutation in the tau gene in familial multiple system tauopathy with presenile dementia | Q36507684 | ||
| Clinicopathologic correlation in PGRN mutations | Q36533936 | ||
| Quantitative classification of primary progressive aphasia at early and mild impairment stages | Q36623393 | ||
| Pathogenic implications of mutations in the tau gene in pallido-ponto-nigral degeneration and related neurodegenerative disorders linked to chromosome 17 | Q36637691 | ||
| Disturbance of nuclear and cytoplasmic TAR DNA-binding protein (TDP-43) induces disease-like redistribution, sequestration, and aggregate formation | Q36741538 | ||
| Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice | Q36788842 | ||
| Neurodegeneration in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9orf72 is linked to TDP-43 pathology and not associated with aggregated forms of dipeptide repeat proteins. | Q36799853 | ||
| Frontotemporal lobar degeneration: clinical and pathological relationships | Q36849424 | ||
| Prevalence, characteristics, and survival of frontotemporal lobar degeneration syndromes | Q36862751 | ||
| Dendritic mRNA: transport, translation and function | Q36937891 | ||
| A distinct clinical, neuropsychological and radiological phenotype is associated with progranulin gene mutations in a large UK series | Q36960858 | ||
| Multiple roles of TDP-43 in gene expression, splicing regulation, and human disease. | Q36991962 | ||
| Co-Occurrence of Language and Behavioural Change in Frontotemporal Lobar Degeneration | Q37020786 | ||
| Links between frontotemporal lobar degeneration, corticobasal degeneration, progressive supranuclear palsy, and amyotrophic lateral sclerosis | Q37036218 | ||
| Accumulation of dipeptide repeat proteins predates that of TDP-43 in frontotemporal lobar degeneration associated with hexanucleotide repeat expansions in C9ORF72 gene | Q37068280 | ||
| Frontotemporal dementia: clinicopathological correlations | Q37069848 | ||
| C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins | Q37094833 | ||
| The anatomic correlate of prosopagnosia in semantic dementia | Q37179032 | ||
| The frontotemporal dementias in a tertiary referral center: classification and demographic characteristics in a series of 232 cases | Q38472488 | ||
| The clinical diagnosis of early-onset dementias: diagnostic accuracy and clinicopathological relationships. | Q38486360 | ||
| Atrophy, hypometabolism and white matter abnormalities in semantic dementia tell a coherent story | Q38489927 | ||
| The most common type of FTLD-FUS (aFTLD-U) is associated with a distinct clinical form of frontotemporal dementia but is not related to mutations in the FUS gene | Q38493216 | ||
| The frontotemporal dementia-motor neuron disease continuum | Q38778723 | ||
| Estimating the number of persons with frontotemporal lobar degeneration in the US population | Q38939262 | ||
| The L266V tau mutation is associated with frontotemporal dementia and Pick-like 3R and 4R tauopathy | Q39071728 | ||
| Prion-like properties of pathological TDP-43 aggregates from diseased brains | Q39129644 | ||
| Conversion of proepithelin to epithelins: roles of SLPI and elastase in host defense and wound repair | Q40677795 | ||
| Progranulin is a mediator of the wound response | Q40678214 | ||
| Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations | Q41942745 | ||
| C9orf72 FTLD/ALS-associated Gly-Ala dipeptide repeat proteins cause neuronal toxicity and Unc119 sequestration | Q42003533 | ||
| Semantic dementia, progressive non-fluent aphasia and their association with amyotrophic lateral sclerosis | Q42340459 | ||
| Frontotemporal and motor neurone degeneration with neurofilament inclusion bodies: additional evidence for overlap between FTD and ALS. | Q42442434 | ||
| Neurofilament inclusion body disease: a new proteinopathy? | Q42444985 | ||
| Late-onset frontotemporal dementia with a novel exon 1 (Arg5His) tau gene mutation | Q43938468 | ||
| Bidirectional transcripts of the expanded C9orf72 hexanucleotide repeat are translated into aggregating dipeptide repeat proteins | Q46797980 | ||
| Dipeptide repeat protein pathology in C9ORF72 mutation cases: clinico-pathological correlations | Q46986726 | ||
| Early dipeptide repeat pathology in a frontotemporal dementia kindred with C9ORF72 mutation and intellectual disability | Q47548592 | ||
| Progressive anomia revisited: focal degeneration associated with progranulin gene mutation | Q47562927 | ||
| A case of sporadic Pick disease with onset at 27 years | Q48091174 | ||
| Distinct clinical characteristics of C9orf72 expansion carriers compared with GRN, MAPT, and nonmutation carriers in a Flanders-Belgian FTLD cohort | Q48200005 | ||
| Neuropsychiatric studies in a family with presenile dementia different from Alzheimer and Pick disease | Q48209616 | ||
| Frontotemporal dementia in The Netherlands: patient characteristics and prevalence estimates from a population-based study | Q48247819 | ||
| Survival in progressive supranuclear palsy and frontotemporal dementia | Q48251313 | ||
| Patients with a novel neurofilamentopathy: dementia with neurofilament inclusions | Q48334782 | ||
| Mutation-specific functional impairments in distinct tau isoforms of hereditary FTDP-17. | Q48338403 | ||
| Molecular analysis and biochemical classification of TDP-43 proteinopathy | Q48350651 | ||
| The neuropathology of frontotemporal lobar degeneration caused by mutations in the progranulin gene | Q48384348 | ||
| Demographic, neurological and behavioural characteristics and brain perfusion SPECT in frontal variant of frontotemporal dementia. | Q48384360 | ||
| Autopsy-proven, sporadic pick disease with onset at age 25 years | Q48602972 | ||
| An immunohistochemical study of cases of sporadic and inherited frontotemporal lobar degeneration using 3R- and 4R-specific tau monoclonal antibodies | Q48609551 | ||
| Clinical features of frontotemporal dementia due to the intronic tau 10(+16) mutation. | Q48623827 | ||
| Pick's disease associated with the novel Tau gene mutation K369I. | Q48744508 | ||
| Behaviour in frontotemporal dementia, Alzheimer's disease and vascular dementia | Q48831565 | ||
| Immunohistochemical identification of messenger RNA-related proteins in basophilic inclusions of adult-onset atypical motor neuron disease | Q49055972 | ||
| Clinicopathologic analysis of frontotemporal and corticobasal degenerations and PSP. | Q50414738 | ||
| Cognitive-behavioural features of progressive supranuclear palsy syndrome overlap with frontotemporal dementia. | Q50597996 | ||
| Phenotype variability in progranulin mutation carriers: a clinical, neuropsychological, imaging and genetic study. | Q51967426 | ||
| Phenotypic variability associated with progranulin haploinsufficiency in patients with the common 1477C-->T (Arg493X) mutation: an international initiative. | Q51971909 | ||
| A 30-unit hexanucleotide repeat expansion in C9orf72 induces pathological lesions with dipeptide-repeat proteins and RNA foci, but not TDP-43 inclusions and clinical disease. | Q52149799 | ||
| Accelerated filament formation from tau protein with specific FTDP-17 missense mutations. | Q53228050 | ||
| Tau gene mutation G389R causes a tauopathy with abundant pick body-like inclusions and axonal deposits. | Q53338420 | ||
| Frontotemporal dementia associated with the C9ORF72 mutation: a unique clinical profile | Q56770874 | ||
| Survival in two variants of tau-negative frontotemporal lobar degeneration: FTLD-U vs FTLD-MND | Q57306390 | ||
| The clinical and neuroanatomical phenotype of FUS associated frontotemporal lobar degeneration | Q57384536 | ||
| P921 | main subject | phenotype | Q104053 |
| frontotemporal lobar degeneration | Q18579 | ||
| P577 | publication date | 2017-01-18 | |
| P1433 | published in | Brain Pathology | Q4955776 |
| P1476 | title | Frontotemporal lobar degeneration: Pathogenesis, pathology and pathways to phenotype |
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