FB2025_05 , released December 11, 2025
FB2025_05 , released December 11, 2025
CV Term Report
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General Information
Term inclusion body myopathy with Paget disease of bone and frontotemporal dementia ID (Ontology) DOID:0050881 (Human Disease)
Definition A syndrome that is characterized by progressive proximal muscle weakness, steolytic bone lesions consistent with Paget disease, and frontotemporal dementia and has_material_basis_in mutation in the valosin containing protein.
Also Known As "IBMPFD" ; "inclusion body myopathy with Paget's disease of bone and frontotemporal dementia"
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Data Class Field Records
Alleles (FBal)  HUMAN_DISEASE_INTERACTIONS      17
Human Disease Models (FBhh)  DOID       2
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Relevant FlyBase reports
 Alleles Genes Human Disease Models
 inclusion body myopathy with Paget disease of bone and frontotemporal dementia      17      9      2
 ameliorates | inclusion body myopathy with Paget disease of bone and frontotemporal dementia       8       --       --
 exacerbates | inclusion body myopathy with Paget disease of bone and frontotemporal dementia       2       --       --
 model of | inclusion body myopathy with Paget disease of bone and frontotemporal dementia       7       --       --
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  disease
   |__syndrome
       |__inclusion body myopathy with Paget disease of bone and frontotemporal dementia  53 rec.
           |__inclusion body myopathy and brain white matter abnormalities 2 rec.
           |__inclusion body myopathy with early-onset Paget disease of bone with or without frontotemporal dementia 1 15 rec.
           |__inclusion body myopathy with early-onset Paget disease of bone with or without frontotemporal dementia 2 13 rec.
           |__inclusion body myopathy with early-onset Paget disease of bone with or without frontotemporal dementia 3 5 rec.
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Synonyms
  • "IBMPFD" EXACT OMO:0003012
    "inclusion body myopathy with Paget's disease of bone and frontotemporal dementia" EXACT
Secondary IDs
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MESH:C563476
MIM:PS167320
ORDO:52430