Term Name: | Huntington's disease-like 1 |
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Synonyms: | autosomal dominant Huntington-like neurodegenerative disorder, early-onset prion disease with prominent psychiatric features, HDL1, HLN1, Huntington disease-like 1, Huntington-like neurodegenerative disorder 1 |
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. |
Ontology: | Human Disease [DOID:0090103] ( DOID:0090103 ) |