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@ARTICLE{Davidson:139228,
      author       = {Davidson, Yvonne S and Flood, Louis and Robinson, Andrew C
                      and Nihei, Yoshihiro and Mori, Kohji and Rollinson, Sara and
                      Richardson, Anna and Benson, Bridget C and Jones, Matthew
                      and Snowden, Julie S and Pickering-Brown, Stuart and Haass,
                      Christian and Lashley, Tammaryn and Mann, David M A},
      title        = {{H}eterogeneous ribonuclear protein {A}3 (hn{RNP} {A}3) is
                      present in dipeptide repeat protein containing inclusions in
                      {F}rontotemporal {L}obar {D}egeneration and {M}otor
                      {N}eurone disease associated with expansions in {C}9orf72
                      gene.},
      journal      = {Acta Neuropathologica Communications},
      volume       = {5},
      number       = {1},
      issn         = {2051-5960},
      address      = {London},
      publisher    = {Biomed Central},
      reportid     = {DZNE-2020-05550},
      pages        = {31},
      year         = {2017},
      abstract     = {Frontotemporal Lobar Degeneration (FTLD) encompasses
                      certain related neurodegenerative disorders which alter
                      behaviour, personality and language. Heterogeneous
                      ribonuclear proteins (hnRNPs) maintain RNA metabolism and
                      changes in their function may underpin the pathogenesis of
                      FTLD. Immunostaining for hnRNP A1, A2/B1 and A3 was
                      performed on sections of temporal cortex with hippocampus
                      from 61 patients with FTLD, stratified by pathological
                      hallmarks into FTLD-tau and FTLD-TDP type A, B and C
                      subtypes, and by genetics into patients with C9orf72
                      expansions, MAPT or GRN mutations, or those without known
                      mutation. Four patients with Motor Neurone Disease (MND)
                      with C9orf72 expansions and 10 healthy controls were also
                      studied. Semi-quantitative analysis assessed hnRNP staining
                      intensity in dentate gyrus (DG) and CA4 region of
                      hippocampus, and temporal cortex (Tcx) in the different
                      pathological and genetic groups.Immunostaining for hnRNP A1,
                      A2/B1 and A3 revealed no consistent changes in pattern or
                      amount of physiological staining across any of the
                      pathological or genetic groups. No immunostaining of any
                      inclusions resembling TDP-43 immunoreactive neuronal
                      cytoplasmic inclusions or dystrophic neurites, was seen in
                      either Tcx or DG of the hippocampus in any of the FTLD cases
                      investigated for hnRNP A1, A2/B1 and A3. However,
                      immunostaining for hnRNP A3 showed that inclusion bodies,
                      resembling those TDP-43 negative, p62-immunopositive
                      structures containing dipeptide repeat proteins (DPR) were
                      variably observed in hippocampus and cerebellum. The
                      proportion of cases showing hnRNP A3-immunoreactive DPR, and
                      the number of hnRNP A3-positive inclusions within cases, was
                      significantly greater in DG than in cells of CA4 region and
                      cerebellum, but the latter was significantly less in all
                      three regions compared to that detected by p62
                      immunostaining.},
      keywords     = {Aged / Aged, 80 and over / C9orf72 Protein: genetics /
                      Cerebellum: metabolism / Cerebellum: pathology / DNA Repeat
                      Expansion / DNA-Binding Proteins: metabolism / Female /
                      Frontotemporal Lobar Degeneration: genetics / Frontotemporal
                      Lobar Degeneration: metabolism / Frontotemporal Lobar
                      Degeneration: pathology / Heterogeneous Nuclear
                      Ribonucleoprotein A1: metabolism / Heterogeneous-Nuclear
                      Ribonucleoprotein Group A-B: metabolism / Hippocampus:
                      metabolism / Hippocampus: pathology / Humans / Inclusion
                      Bodies: metabolism / Inclusion Bodies: pathology /
                      Intercellular Signaling Peptides and Proteins: genetics /
                      Male / Middle Aged / Motor Neuron Disease: genetics / Motor
                      Neuron Disease: metabolism / Motor Neuron Disease: pathology
                      / Progranulins / Temporal Lobe: metabolism / Temporal Lobe:
                      pathology / tau Proteins: genetics / C9orf72 Protein (NLM
                      Chemicals) / C9orf72 protein, human (NLM Chemicals) /
                      DNA-Binding Proteins (NLM Chemicals) / GRN protein, human
                      (NLM Chemicals) / HNRNPA3 protein, human (NLM Chemicals) /
                      Heterogeneous Nuclear Ribonucleoprotein A1 (NLM Chemicals) /
                      Heterogeneous-Nuclear Ribonucleoprotein Group A-B (NLM
                      Chemicals) / Intercellular Signaling Peptides and Proteins
                      (NLM Chemicals) / MAPT protein, human (NLM Chemicals) /
                      Progranulins (NLM Chemicals) / TDP-43 protein, human (NLM
                      Chemicals) / hnRNP A2 (NLM Chemicals) / tau Proteins (NLM
                      Chemicals)},
      cin          = {AG Haass},
      ddc          = {610},
      cid          = {I:(DE-2719)1110007},
      pnm          = {342 - Disease Mechanisms and Model Systems (POF3-342)},
      pid          = {G:(DE-HGF)POF3-342},
      typ          = {PUB:(DE-HGF)16},
      pubmed       = {pmid:28431575},
      pmc          = {pmc:PMC5399321},
      doi          = {10.1186/s40478-017-0437-5},
      url          = {https://pub.dzne.de/record/139228},
}