{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,28]],"date-time":"2026-08-28T05:25:55Z","timestamp":1787894755715,"version":"build-2784847793"},"reference-count":98,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2021,5,27]],"date-time":"2021-05-27T00:00:00Z","timestamp":1622073600000},"content-version":"vor","delay-in-days":56,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Motor Neuron Disease Association, the Medical Research Council UK"},{"name":"Lady Edith Wolfson Fellowship Fund, the van Geest Foundation, the Rosetrees Trust (M799), Alzheimer\u2019s Research UK","award":["ARUK-PG2018B-008"],"award-info":[{"award-number":["ARUK-PG2018B-008"]}]},{"DOI":"10.13039\/100016210","name":"Psychiatry Research Trust","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100016210","id-type":"DOI","asserted-by":"publisher"}]},{"name":"van Geest Foundation"},{"DOI":"10.13039\/501100004543","name":"Chinese Scholarship Council","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100004543","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Alzheimer\u2019s Research UK King\u2019s College London Network Centre"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,4,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Amyotrophic lateral sclerosis and frontotemporal dementia are overlapping diseases in which MRI reveals brain structural changes in advance of symptom onset. Recapitulating these changes in preclinical models would help to improve our understanding of the molecular causes underlying regionally selective brain atrophy in early disease. We therefore investigated the translational potential of the TDP-43Q331K knock-in mouse model of amyotrophic lateral sclerosis-frontotemporal dementia using MRI. We performed in vivo MRI of TDP-43Q331K knock-in mice. Regions of significant volume change were chosen for post-mortem brain tissue analyses. Ex vivo computed tomography was performed to investigate skull shape. Parvalbumin neuron density was quantified in post-mortem amyotrophic lateral sclerosis frontal cortex. Adult mutants demonstrated parenchymal volume reductions affecting the frontal lobe and entorhinal cortex in a manner reminiscent of amyotrophic lateral sclerosis-frontotemporal dementia. Subcortical, cerebellar and brain stem regions were also affected in line with observations in pre-symptomatic carriers of mutations in C9orf72, the commonest genetic cause of both amyotrophic lateral sclerosis and frontotemporal dementia. Volume loss was also observed in the dentate gyrus of the hippocampus, along with ventricular enlargement. Immunohistochemistry revealed reduced parvalbumin interneurons as a potential cellular correlate of MRI changes in mutant mice. By contrast, microglia was in a disease activated state even in the absence of brain volume loss. A reduction in immature neurons was found in the dentate gyrus, indicative of impaired adult neurogenesis, while a paucity of parvalbumin interneurons in P14 mutant mice suggests that TDP-43Q331K disrupts neurodevelopment. Computerized tomography imaging showed altered skull morphology in mutants, further suggesting a role for TDP-43Q331K in development. Finally, analysis of human post-mortem brains confirmed a paucity of parvalbumin interneurons in the prefrontal cortex in sporadic amyotrophic lateral sclerosis and amyotrophic lateral sclerosis linked to C9orf72 mutations. Regional brain MRI changes seen in human amyotrophic lateral sclerosis-frontotemporal dementia are recapitulated in TDP-43Q331K knock-in mice. By marrying in vivo imaging with targeted histology, we can unravel cellular and molecular processes underlying selective brain vulnerability in human disease. As well as helping to understand the earliest causes of disease, our MRI and histological markers will be valuable in assessing the efficacy of putative therapeutics in TDP-43Q331K knock-in mice.<\/jats:p>","DOI":"10.1093\/braincomms\/fcab114","type":"journal-article","created":{"date-parts":[[2021,5,26]],"date-time":"2021-05-26T07:29:05Z","timestamp":1622014145000},"source":"Crossref","is-referenced-by-count":23,"title":["MRI-guided histology of TDP-43 knock-in mice implicates parvalbumin interneuron loss, impaired neurogenesis and aberrant neurodevelopment in amyotrophic lateral sclerosis-frontotemporal dementia"],"prefix":"10.1093","volume":"3","author":[{"given":"Ziqiang","family":"Lin","sequence":"first","affiliation":[{"name":"Department of Basic and Clinical Neuroscience, The Maurice Wohl Clinical Neuroscience Institute, Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King\u2019s College London, London SE5 9RT, UK"},{"name":"West China School of Medicine, West China Hospital, Sichuan University, Chengdu 610041, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eugene","family":"Kim","sequence":"additional","affiliation":[{"name":"BRAIN Centre (Biomarker Research And Imaging for Neuroscience), Department of Neuroimaging, IoPPN, King\u2019s College London, London SE5 9NU, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohi","family":"Ahmed","sequence":"additional","affiliation":[{"name":"Centre for Craniofacial and Regenerative Biology, Floor 27 Tower Wing, Guy\u2019s Hospital, King\u2019s College London, London SE1 9RT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gang","family":"Han","sequence":"additional","affiliation":[{"name":"Molecular Neurobiology Group, Institute of Life Sciences, School of Medicine, Swansea University, Swansea SA2 8PP, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Camilla","family":"Simmons","sequence":"additional","affiliation":[{"name":"BRAIN Centre (Biomarker Research And Imaging for Neuroscience), Department of Neuroimaging, IoPPN, King\u2019s College London, London SE5 9NU, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yushi","family":"Redhead","sequence":"additional","affiliation":[{"name":"Centre for Craniofacial and Regenerative Biology, Floor 27 Tower Wing, Guy\u2019s Hospital, King\u2019s College London, London SE1 9RT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2593-5270","authenticated-orcid":false,"given":"Jack","family":"Bartlett","sequence":"additional","affiliation":[{"name":"Molecular Neurobiology Group, Institute of Life Sciences, School of Medicine, Swansea University, Swansea SA2 8PP, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luis Emiliano","family":"Pena Altamira","sequence":"additional","affiliation":[{"name":"Department of Basic and Clinical Neuroscience, The Maurice Wohl Clinical Neuroscience Institute, Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King\u2019s College London, London SE5 9RT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isobel","family":"Callaghan","sequence":"additional","affiliation":[{"name":"Department of Basic and Clinical Neuroscience, The Maurice Wohl Clinical Neuroscience Institute, Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King\u2019s College London, London SE5 9RT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4147-4267","authenticated-orcid":false,"given":"Matthew A","family":"White","sequence":"additional","affiliation":[{"name":"Department of Basic and Clinical Neuroscience, The Maurice Wohl Clinical Neuroscience Institute, Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King\u2019s College London, London SE5 9RT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nisha","family":"Singh","sequence":"additional","affiliation":[{"name":"BRAIN Centre (Biomarker Research And Imaging for Neuroscience), Department of Neuroimaging, IoPPN, King\u2019s College London, London SE5 9NU, UK"},{"name":"School of Biomedical Engineering & Imaging Sciences, St Thomas' Hospital, King's College London, 4th floor Lambeth Wing, London SE1 7EH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen","family":"Sawiak","sequence":"additional","affiliation":[{"name":"Department of Clinical Neurosciences, Cambridge University, Cambridge CB2 0QQ, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2530-0598","authenticated-orcid":false,"given":"Tara","family":"Spires-Jones","sequence":"additional","affiliation":[{"name":"Centre for Discovery Brain Sciences, University of Edinburgh, Edinburgh EH8 9XD, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony C","family":"Vernon","sequence":"additional","affiliation":[{"name":"Department of Basic and Clinical Neuroscience, The Maurice Wohl Clinical Neuroscience Institute, Institute of Psychiatry, Psychology and Neuroscience (IoPPN), King\u2019s College London, London SE5 9RT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael P","family":"Coleman","sequence":"additional","affiliation":[{"name":"Brain Repair Centre, Cambridge University, Cambridge CB2 0PY, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeremy","family":"Green","sequence":"additional","affiliation":[{"name":"Centre for Craniofacial and Regenerative Biology, Floor 27 Tower Wing, Guy\u2019s Hospital, King\u2019s College London, London SE1 9RT, 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