{
  "story_id": "ea0af9439dce38ee052cb4c69dc410a0",
  "desk": "drm3",
  "revision": 1,
  "published_at": "2026-09-03T20:45:56.000Z",
  "content_hash": "6402e9128948f37bbf579fde2ff34354224982b9cbf72a7a242fef56025891ed",
  "hash_basis": "sha256 over `headline\\ndek\\nprose`, plus `\\n` + the canonical citations JSON when any source is placed, plus `\\n#blog` for blogs",
  "basis": {
    "headline": "FDA Approves Zanvastro as First Treatment for Alexander Disease",
    "dek": "FDA approves Zanvastro, the first therapy targeting the underlying cause of Alexander disease.",
    "prose": "The U.S. Food and Drug Administration approved Zanvastro (zilganersen) injection for the treatment of Alexander disease in pediatric and adult patients on September 03, 2026. [^1]\n\nIonis Pharmaceuticals received FDA approval on September 3, 2026, for Zanvastro, the first disease-modifying therapy for Alexander disease. [^2]\n\nAlexander disease is a rare, progressive neurological disorder caused by mutations in the gene that produces glial fibrillary acidic protein (GFAP), affecting less than 1 in a million people. [^3]\n\nA pivotal trial showed that walking speed stayed stable in treated patients while the control group saw a 33% decline. [^4]\n\nResearchers found hints that treating young children can improve motor function, not just stabilize it. [^5]",
    "cited": "[{\"statement\":\"The U.S. Food and Drug Administration approved Zanvastro (zilganersen) injection for the treatment of Alexander disease in pediatric and adult patients on September 03, 2026.\",\"source\":\"FDA\",\"instrument\":\"News\",\"claim_key\":null,\"published_at\":\"2026-09-03T19:48:27.000Z\",\"publisher_count\":1,\"sources\":[\"FDA\"]},{\"statement\":\"Ionis Pharmaceuticals received FDA approval on September 3, 2026, for Zanvastro, the first disease-modifying therapy for Alexander disease.\",\"source\":\"STAT\",\"instrument\":\"News\",\"claim_key\":null,\"published_at\":\"2026-09-03T20:45:56.000Z\",\"publisher_count\":1,\"sources\":[\"STAT\"]},{\"statement\":\"Alexander disease is a rare, progressive neurological disorder caused by mutations in the gene that produces glial fibrillary acidic protein (GFAP), affecting less than 1 in a million people.\",\"source\":\"FDA\",\"instrument\":\"News\",\"claim_key\":null,\"published_at\":\"2026-09-03T19:48:27.000Z\",\"publisher_count\":1,\"sources\":[\"FDA\"]},{\"statement\":\"A pivotal trial showed that walking speed stayed stable in treated patients while the control group saw a 33% decline.\",\"source\":\"STAT\",\"instrument\":\"News\",\"claim_key\":null,\"published_at\":\"2026-09-03T20:45:56.000Z\",\"publisher_count\":1,\"sources\":[\"STAT\"]},{\"statement\":\"Researchers found hints that treating young children can improve motor function, not just stabilize it.\",\"source\":\"STAT\",\"instrument\":\"News\",\"claim_key\":null,\"published_at\":\"2026-09-03T20:45:56.000Z\",\"publisher_count\":1,\"sources\":[\"STAT\"]}]",
    "kind": "news"
  },
  "receipt_verify": "Ed25519 over the dot-joined string `slice_hash.cursor_from.cursor_to.view.view_version.row_count`; public_key and sig are base64url of the raw 32-byte key / 64-byte signature",
  "receipt": null,
  "receipt_note": "this revision predates receipt-keeping (before v0.37.0); the filed row lives in the record",
  "generation_chain": {
    "wire": {
      "stream": "fountain_news",
      "story_id": "2caf2bc1b671baf441dcc0a40eb4f408",
      "thread_id": "1c3ded46d054bbb3124727a871b03aaa",
      "thread_label": "Alexander disease",
      "novelty": "UPDATE",
      "content_hash": "39a63cd64a1e84558ae7815cf41e1d49c558ef0b38297b700867188ae25888ad",
      "last_published_at": "2026-09-03T20:45:56.000Z",
      "read_receipt": {
        "slice_hash": "3528f573095b38c4abfe7176deb5e225e82c1af3c7dc91680410a8795d94b55d",
        "cursor_from": "eyJ0cyI6IjIwMjYtMDktMDNUMjA6MTc6MDAuMDAwMDAwWiIsImlkIjoiNWI1MmIxMjk3M2NhODBmY2NiMjk5ZDgyMWQ5MjljZmEiLCJ2IjoiMSJ9",
        "cursor_to": "eyJ0cyI6IjIwMjYtMDktMDNUMjA6NTA6MzEuMDAwMDAwWiIsImlkIjoiNTFkOTVlNTZjYmQ0MGI5YmU1MWVjMjY3ZjU4ZWI2YWEiLCJ2IjoiMSJ9",
        "view": "v_fountain_news",
        "view_version": "1",
        "row_count": 100,
        "window_days": 3,
        "bytes_scanned": 12166856,
        "credits": 8,
        "price_per_100_rows": 8,
        "sig": "4tF4JvaY1J1xDk_C-HWRYlI54NluY8U5eQ9TCeC5PhJa45GX2XouLN6UhzZTcJYQ7Ko6oaJw6PLT38LzBV4_Aw",
        "public_key": "bMUigy8O0jOnBxQ4Sc-5lwhIZ8LQVAhxMbR7qESVuUE",
        "signer_path": "lakehouse/data-extract/v1",
        "alg": "Ed25519",
        "signed": true
      }
    },
    "written_at": "2026-09-04T06:39:57.276Z"
  },
  "cited_facts": [
    {
      "statement": "The U.S. Food and Drug Administration approved Zanvastro (zilganersen) injection for the treatment of Alexander disease in pediatric and adult patients on September 03, 2026.",
      "source": "FDA",
      "instrument": "News",
      "claim_key": null,
      "published_at": "2026-09-03T19:48:27.000Z",
      "publisher_count": 1,
      "sources": [
        "FDA"
      ]
    },
    {
      "statement": "Ionis Pharmaceuticals received FDA approval on September 3, 2026, for Zanvastro, the first disease-modifying therapy for Alexander disease.",
      "source": "STAT",
      "instrument": "News",
      "claim_key": null,
      "published_at": "2026-09-03T20:45:56.000Z",
      "publisher_count": 1,
      "sources": [
        "STAT"
      ]
    },
    {
      "statement": "Alexander disease is a rare, progressive neurological disorder caused by mutations in the gene that produces glial fibrillary acidic protein (GFAP), affecting less than 1 in a million people.",
      "source": "FDA",
      "instrument": "News",
      "claim_key": null,
      "published_at": "2026-09-03T19:48:27.000Z",
      "publisher_count": 1,
      "sources": [
        "FDA"
      ]
    },
    {
      "statement": "A pivotal trial showed that walking speed stayed stable in treated patients while the control group saw a 33% decline.",
      "source": "STAT",
      "instrument": "News",
      "claim_key": null,
      "published_at": "2026-09-03T20:45:56.000Z",
      "publisher_count": 1,
      "sources": [
        "STAT"
      ]
    },
    {
      "statement": "Researchers found hints that treating young children can improve motor function, not just stabilize it.",
      "source": "STAT",
      "instrument": "News",
      "claim_key": null,
      "published_at": "2026-09-03T20:45:56.000Z",
      "publisher_count": 1,
      "sources": [
        "STAT"
      ]
    }
  ],
  "note": "A signature proves who filed this and that it has not changed since. It never makes a claim true."
}