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Task D-005 · Type D — Disease → mechanism

How does CAG repeat expansion in the HTT gene lead to neurodegeneration in Huntington disease?

Difficulty
medium
Rarity stratum
high
Evidence type
primary_research + case_report
MONDO ID
MONDO:0007739
Curator notes

The corpus documents describe the polyglutamine-expansion mechanism and downstream GABAergic neuron loss at a fairly high level; a fully mechanistic answer (e.g. specific proteostasis/autophagy pathway details) would need documents outside this 24-document v0 sample — flagged as a partial-coverage task, not a full-mechanism one.

Generation outcome

Abstained
No
Abstention correct
Yes
Gold in retrieved
2.00
Generation cost
$0.000437
Claims
5
Supported
5
Contradicted
0
Unverifiable
0

Gold documents (2)

  • Poly ADP-ribose signaling is dysregulated in Huntington disease.
    pubmed:39331414retrieved ✓source
  • Huntington's Disease: A Report of an Interesting Case and Literature Review.
    pubmed:38567236retrieved ✓source

Retrieved documents (6)

  • CSPα in neurodegenerative diseases.
    pubmed:36466613
  • Poly ADP-ribose signaling is dysregulated in Huntington disease.
    pubmed:39331414gold
  • Huntington's Disease: A Report of an Interesting Case and Literature Review.
    pubmed:38567236gold
  • Translating HDAC inhibitors in Friedreich's ataxia.
    pubmed:28392990
  • Friedreich's ataxia: new insights.
    pubmed:37698160
  • Autophagy and Polyglutamine Disease.
    pubmed:32671744

Generated claims (5)

supportedconfidence 0.95

CAG repeat expansion in the HTT gene causes an expanded polyglutamine tract in the HTT protein.

pubmed:39331414
supportedconfidence 0.90

The age of onset and severity of Huntington disease are related to the length of the polyglutamine fragment.

pubmed:32671744
supportedconfidence 0.95

CAG repeat expansion in the HTT gene affects DNA repair pathways, particularly the PARP pathway.

pubmed:39331414
supportedconfidence 0.90

Mutant HTT protein impairs the PAR response and contributes to neuronal dysfunction.

pubmed:39331414
supportedconfidence 0.95

CAG repeat expansion in the HTT gene results in the progressive deterioration of GABAergic neurons in the basal ganglia.

pubmed:38567236