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Human Disease and Mouse Model Detail
Human Disease Inclusion Body Myopathy with Early-Onset Paget Disease with or without Frontotemporal Dementia 1; IBMPFD1
OMIM ID: 167320
Synonyms Lower Motor Neuron Degeneration with Paget-Like Bone Disease; Multisystem Proteinopathy 1; MSP1; Muscular Dystrophy, Limb-Girdle, with Paget Disease of Bone; Myopathy, Inclusion Body; Paget Disease of Bone; Pagetoid Amyotrophic Lateral Sclerosis; Pagetoid Neuroskeletal Syndrome
View all models View ALL (5) mouse models for this human disease.
Genes and
mouse models
Mutations in human and/or mouse homologs are associated with this disease

      *Gene is associated with the disease in this species
      Mouse Homologs Human Homologs Mouse Models Mouse : Human
Homology Class
     Vcp* VCP* View 2 models 1:1 Homology
Transgenes and
other genome features
Transgenes and other genome features developed in mice to model this disease.

     Transgenes and Other Genome Features Mouse Models
  Tg(CAG-VCP*A232E)93Jpat View 1 model
  Tg(CAG-VCP*R155H)55Jpat View 1 model
  Tg(Thy1-VCP*A232E)BMaki View 1 model

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
MGI 5.17
The Jackson Laboratory