FB2025_05 , released December 11, 2025
FB2025_05 , released December 11, 2025
CV Term Report
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General Information
Term Huntington's disease-like 1 ID (Ontology) DOID:0090103 (Human Disease)
Definition A prion disease that is characterized by a phenocopy of Huntington disease (unwanted choreatic movements, behavioral and psychiatric disturbances and dementia) that has_material_basis_in autosomal dominant inheritance of 8 extra octapeptide repeats in the prion protein (PRNP) gene on chromosome 20p13.
Also Known As "autosomal dominant Huntington-like neurodegenerative disorder" ; "early-onset prion disease with prominent psychiatric features" ; "HDL1" (for all, see Synonyms field below)
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Spanning Tree (Parents/Children)
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disease by infectious agent__
brain disease________________|
                             prion disease
                              |__Huntington's disease-like 1
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Synonyms
  • "autosomal dominant Huntington-like neurodegenerative disorder" EXACT
    "early-onset prion disease with prominent psychiatric features" EXACT
    "HDL1" EXACT OMO:0003012
    "HLN1" EXACT OMO:0003012
    "Huntington disease-like 1" EXACT
    "Huntington-like neurodegenerative disorder 1" EXACT
Secondary IDs
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ICD10CM:G10
MIM:603218
ORDO:157941