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Task H-010 · Type H — Insufficient evidence (should abstain)

What evidence exists for the investigational gene therapy PBGM01 in Krabbe disease?

Difficulty
medium
Rarity stratum
sparse
Evidence type
insufficient_evidence
MONDO ID
MONDO:0009499
Curator notes

VERIFIED by full-text grep of the Krabbe disease document subset (2026-09-08): "PBGM01" does not appear, even though the corpus DOES contain a different Krabbe gene therapy paper (pubmed:35333110) — this makes the task harder than a topic-blind absence: the model must distinguish "gene therapy exists in the corpus for this disease" from "THIS SPECIFIC investigational product is in the corpus," a sharper test of citation precision than H-tasks where the whole topic is absent.

Generation outcome

Abstained
No
Abstention correct
No
Gold in retrieved
n/a (no gold docs)
Generation cost
$0.000451
Claims
3
Supported
2
Contradicted
1
Unverifiable
0

Gold documents (0)

No gold documents — this is an insufficient-evidence (Type H) task; the correct answer is abstention.

Retrieved documents (6)

  • Krabbe's disease; A rare case report.
    pubmed:36272395
  • Globoid cell leukodystrophy (Krabbe disease).
    pubmed:42554324
  • Brain accumulation of lactosylceramide characterizes GALC deficiency in a zebrafish model of Krabbe disease.
    pubmed:40305757
  • Krabbe disease--clinical profile.
    pubmed:10992329
  • Clinical and molecular findings in 6 Turkish cases with Krabbe disease.
    pubmed:35286032
  • Efficacy and Safety of a Krabbe Disease Gene Therapy.
    pubmed:35333110

Generated claims (3)

contradictedconfidence 0.90

PBGM01 has been shown to improve survival in animal models of Krabbe disease.

pubmed:35333110
supportedconfidence 0.85

PBGM01 treatment prevents psychosine accumulation and preserves peripheral nerve myelination in dogs with Krabbe disease.

pubmed:35333110
supportedconfidence 0.90

PBGM01 was tested in nonhuman primates without dose-limiting toxicity.

pubmed:35333110