{
  "abstract": "Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by progressive degeneration of upper and lower motor neurons. Cytoplasmic accumulation of phosphorylated TAR DNA-binding protein 43 (pTDP-43) is the pathological hallmark of most ALS cases. While ALS has traditionally been viewed as a disease confined to the brain, spinal cord and motor nerves, recent studies have reported pTDP-43 pathology in multiple other tissues (skeletal and cardiac muscle, skin, minor salivary glands, gastrointestinal tract and lymph nodes), referred to as peripheral pathology. The detection of pTDP-43 beyond the nervous system suggests that ALS-associated TDP-43 proteinopathy may be more widespread than previously recognized and raises fundamental questions regarding the spatial and temporal landscape of ALS pathology. Peripheral pTDP-43 accumulation may reflect a systemic biological susceptibility affecting multiple tissues, propagation of pathological TDP-43 species between anatomical compartments, or a combination of both mechanisms. While its biological significance is yet to be determined, the presence of pTDP-43 in peripheral tissues broadens the current conceptual framework of ALS. It may also provide new opportunities for pathology-based biomarkers, therapeutic monitoring, and mechanistic studies aimed at understanding disease initiation and progression. However, current evidence is derived from small and methodologically heterogeneous cohorts, and peripheral pTDP-43 pathology is not restricted to ALS, emphasizing the need for larger standardized studies.",
  "abstract_shown": true,
  "affiliations": [],
  "article_type": null,
  "arxiv": null,
  "author_total": 8,
  "authors": [
    {
      "name": "Philippe Codron"
    },
    {
      "name": "Marion Miranda"
    },
    {
      "name": "Maelle Garnier"
    },
    {
      "name": "Shiyang He"
    },
    {
      "name": "Julien Gouju"
    },
    {
      "name": "Julien Cassereau"
    },
    {
      "name": "Pascal Leblanc"
    },
    {
      "name": "Franck Letournel"
    }
  ],
  "bibtex": "https://research.draconitic.com/paper/frrk6tbzv5d6n.bib",
  "cited_by": 0,
  "doi": "10.1007/s00401-026-03086-3",
  "fulltext_url": null,
  "id": "frrk6tbzv5d6n",
  "issns": [
    "0001-6322",
    "1432-0533"
  ],
  "landing_url": "https://hal.science/hal-05790506v1",
  "language": "en",
  "licence": "CC-BY-4.0",
  "licence_label": "CC BY 4.0",
  "licence_source": "article",
  "licence_url": "https://creativecommons.org/licenses/by/4.0/",
  "metadata_licence": null,
  "native_subjects": [
    [
      "hal",
      "0.sdv"
    ],
    [
      "openalex",
      "2728|Amyotrophic Lateral Sclerosis Research"
    ]
  ],
  "openalex": "W7213276150",
  "pdf_url": "https://hal.science/hal-05790506/document",
  "pmcid": null,
  "pmid": null,
  "published": "2026-09-15",
  "publisher": "Springer Verlag",
  "ris": "https://research.draconitic.com/paper/frrk6tbzv5d6n.ris",
  "sources": [
    {
      "first_seen": "2026-10-30T12:17:53Z",
      "native_id": "hal-05790506",
      "source": "hal",
      "url": "https://hal.science/hal-05790506v1"
    }
  ],
  "subjects": [
    {
      "field": "medicine",
      "origin": "openalex:2728|Amyotrophic Lateral Sclerosis Research",
      "subfield": "neurology"
    }
  ],
  "title": "Peripheral TDP-43 pathology in amyotrophic lateral sclerosis: toward a systemic proteinopathy",
  "title_text": "Peripheral TDP-43 pathology in amyotrophic lateral sclerosis: toward a systemic proteinopathy",
  "type": "peer-reviewed",
  "updated_at": "2026-10-30T12:17:53Z",
  "url": "https://research.draconitic.com/paper/frrk6tbzv5d6n",
  "venue": "Acta Neuropathologica",
  "versions": []
}