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article download pdf polyclonal anti-ubq antibody neocortical ubiquitin-immunoreactive inclusions fronto-temporal lobar degeneration inclusion body myopathy cortical ubiquitin-positive inclusions alzheimers research trust motor-neuron disease motor neurone disease frontotemporal lobar degeneration privacy choices/manage cookies neuronal cytoplasmic inclusions pgrn mutation-related ftld amyotrophic lateral sclerosis tau-negative inclusions medical research council neuronal intranuclear inclusions positive family history tau-negative dementia full access intraneuronal cytoplasmic inclusions ubq neuronal inclusions ubiquitin positive inclusions ubq cytoplasmic inclusions previous family history stuart pickering-brown daniel du plessis tau-negative histology pathologically heterogeneous group alternative ubq histologies pickering-brown sm insoluble tau proteins swedish ftdp-17 family intraneuronal neurofibrillary tangles related subjects important nosological implications respiratory failure consequent bigio eh ubiquitinated proteins accumulating ubiquitin-positive pathology total ubq score dentate gyrus numerous conditions privacy policy autosomal dominant inheritance reach statistical significance reached statistical significance autosomal dominant transmission separate underlying pathophysiological canadian family ubc-17 frontal lobe dementia

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         headline:Heterogeneity of ubiquitin pathology in frontotemporal lobar degeneration: classification and relation to clinical phenotype
         description:We have investigated the extent and pattern of immunostaining for ubiquitin protein (UBQ) in 60 patients with frontotemporal lobar degeneration (FTLD) with ubiquitin-positive, tau-negative inclusions (FTLD-U), 37 of whom were ascertained in Manchester UK and 23 in Newcastle-Upon-Tyne, UK. There were three distinct histological patterns according to the form and distribution of the UBQ pathology. Histological type 1 was present in 19 patients (32%) and characterised by the presence of a moderate number, or numerous, UBQ immunoreactive neurites and intraneuronal cytoplasmic inclusions within layer II of the frontal and temporal cerebral cortex, and cytoplasmic inclusions within granule cells of the dentate gyrus; neuronal intranuclear inclusions (NII) of a “cat’s eye” or “lentiform” appearance were present in 17 of these patients. In histological type 2 (16 patients, 27%), UBQ neurites were predominantly, or exclusively, present with few intraneuronal cytoplasmic inclusions within layer II of the cerebral cortex, while in histological type 3 (25 patients, 42%), UBQ intraneuronal cytoplasmic inclusions either within the cortical layer II or in the granule cells of the dentate gyrus, with few or no UBQ neurites, were seen. In neither of these latter two groups were NII present. The influence of histological type on clinical phenotype was highly significant with type 1 histology being associated clinically with cases of frontotemporal dementia (FTD) or progressive non-fluent aphasia (PNFA), type 2 histology with semantic dementia (SD), and type 3 histology with FTD, or FTD and motor neurone disease (MND).
         datePublished:2006-09-26T00:00:00Z
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            Dentate Gyrus
            Motor Neurone Disease
            Cytoplasmic Inclusion
            Semantic Dementia
            Neuronal Cytoplasmic Inclusion
            Pathology
            Neurosciences
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      headline:Heterogeneity of ubiquitin pathology in frontotemporal lobar degeneration: classification and relation to clinical phenotype
      description:We have investigated the extent and pattern of immunostaining for ubiquitin protein (UBQ) in 60 patients with frontotemporal lobar degeneration (FTLD) with ubiquitin-positive, tau-negative inclusions (FTLD-U), 37 of whom were ascertained in Manchester UK and 23 in Newcastle-Upon-Tyne, UK. There were three distinct histological patterns according to the form and distribution of the UBQ pathology. Histological type 1 was present in 19 patients (32%) and characterised by the presence of a moderate number, or numerous, UBQ immunoreactive neurites and intraneuronal cytoplasmic inclusions within layer II of the frontal and temporal cerebral cortex, and cytoplasmic inclusions within granule cells of the dentate gyrus; neuronal intranuclear inclusions (NII) of a “cat’s eye” or “lentiform” appearance were present in 17 of these patients. In histological type 2 (16 patients, 27%), UBQ neurites were predominantly, or exclusively, present with few intraneuronal cytoplasmic inclusions within layer II of the cerebral cortex, while in histological type 3 (25 patients, 42%), UBQ intraneuronal cytoplasmic inclusions either within the cortical layer II or in the granule cells of the dentate gyrus, with few or no UBQ neurites, were seen. In neither of these latter two groups were NII present. The influence of histological type on clinical phenotype was highly significant with type 1 histology being associated clinically with cases of frontotemporal dementia (FTD) or progressive non-fluent aphasia (PNFA), type 2 histology with semantic dementia (SD), and type 3 histology with FTD, or FTD and motor neurone disease (MND).
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         Dentate Gyrus
         Motor Neurone Disease
         Cytoplasmic Inclusion
         Semantic Dementia
         Neuronal Cytoplasmic Inclusion
         Pathology
         Neurosciences
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      name:Department of Neuropathology, Institute for Ageing and Health (IAH), Newcastle General Hospital, Newcastle-Upon-Tyne, UK
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