CONRAD WEIHL

Summary

Affiliation: Washington University School of Medicine
Country: USA

Publications

  1. pmc Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease
    Jeong Sun Ju
    Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA
    J Cell Biol 187:875-88. 2009
  2. pmc Monitoring autophagy in the treatment of protein aggregate diseases: steps toward identifying autophagic biomarkers
    Conrad C Weihl
    Department of Neurology, Washington University School of Medicine, PO Box 8111, 660 South Euclid Avenue, St Louis, MO 63110, USA
    Neurotherapeutics 10:383-90. 2013
  3. pmc Another VCP interactor: NF is enough
    Conrad C Weihl
    Department of Neurology, Washington University School of Medicine, Saint Louis, Missouri 63110, USA
    J Clin Invest 121:4627-30. 2011
  4. pmc Valosin containing protein associated fronto-temporal lobar degeneration: clinical presentation, pathologic features and pathogenesis
    C C Weihl
    Department of Neurology, Washington University School of Medicine, Saint Louis, MO 63110, USA
    Curr Alzheimer Res 8:252-60. 2011
  5. pmc Novel GNE mutations in two phenotypically distinct HIBM2 patients
    Conrad C Weihl
    Department of Neurology and Hope Center for Neurological Disorders, Washington University School of Medicine, 660 S Euclid Avenue, Saint Louis, MO 63110, USA
    Neuromuscul Disord 21:102-5. 2011
  6. pmc Sporadic inclusion body myositis: possible pathogenesis inferred from biomarkers
    Conrad C Weihl
    Department of Neurology and Hope Center for Neurological Disorders, Washington University School of Medicine, 660 S Euclid Avenue, Saint Louis, Missouri 63110, USA
    Curr Opin Neurol 23:482-8. 2010
  7. pmc Valosin-containing protein disease: inclusion body myopathy with Paget's disease of the bone and fronto-temporal dementia
    Conrad C Weihl
    Department of Neurology, Washington University School of Medicine, Saint Louis, MO 63110, USA
    Neuromuscul Disord 19:308-15. 2009
  8. pmc TDP-43 accumulation in inclusion body myopathy muscle suggests a common pathogenic mechanism with frontotemporal dementia
    C C Weihl
    Department of Neurology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Neurol Neurosurg Psychiatry 79:1186-9. 2008
  9. ncbi request reprint Transgenic expression of inclusion body myopathy associated mutant p97/VCP causes weakness and ubiquitinated protein inclusions in mice
    Conrad C Weihl
    Department of Neurology, Washington University School of Medicine, 660 S Euclid Avenue, Saint Louis, MO 63110, USA
    Hum Mol Genet 16:919-28. 2007
  10. ncbi request reprint Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation
    Conrad C Weihl
    Department of Neurology, Washington University School of Medicine, 660 South Euclid Avenue, St Louis, MO 63110, USA
    Hum Mol Genet 15:189-99. 2006

Research Grants

  1. AAA ATPase p97/VCP and Inclusion Body Myopathy
    CONRAD WEIHL; Fiscal Year: 2007
  2. IBMPFD MUTATIONS IMPAIR UPS FUNCTION
    CONRAD WEIHL; Fiscal Year: 2009
  3. IBMPFD MUTATIONS IMPAIR UPS FUNCTION
    Conrad C Weihl; Fiscal Year: 2010
  4. IBMPFD MUTATIONS IMPAIR UPS FUNCTION
    CONRAD WEIHL; Fiscal Year: 2009

Collaborators

Detail Information

Publications10

  1. pmc Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease
    Jeong Sun Ju
    Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA
    J Cell Biol 187:875-88. 2009
    ..These data implicate VCP in autophagy and suggest that impaired autophagy explains the pathology seen in IBMPFD muscle, including TDP-43 accumulation...
  2. pmc Monitoring autophagy in the treatment of protein aggregate diseases: steps toward identifying autophagic biomarkers
    Conrad C Weihl
    Department of Neurology, Washington University School of Medicine, PO Box 8111, 660 South Euclid Avenue, St Louis, MO 63110, USA
    Neurotherapeutics 10:383-90. 2013
    ..These methods will serve as important biomarkers necessary to test compound efficacy and monitor clinical improvement. ..
  3. pmc Another VCP interactor: NF is enough
    Conrad C Weihl
    Department of Neurology, Washington University School of Medicine, Saint Louis, Missouri 63110, USA
    J Clin Invest 121:4627-30. 2011
    ..Thus, aberrant interactions between VCP and NF1 may explain the dementia phenotype and cognitive delay observed in patients with IBMPFD and neurofibromatosis type 1...
  4. pmc Valosin containing protein associated fronto-temporal lobar degeneration: clinical presentation, pathologic features and pathogenesis
    C C Weihl
    Department of Neurology, Washington University School of Medicine, Saint Louis, MO 63110, USA
    Curr Alzheimer Res 8:252-60. 2011
    ..In addition, we will discuss the current understanding regarding VCP's function in terminally differentiated tissue and how disease associated mutations result in both myo- and neurodegeneration...
  5. pmc Novel GNE mutations in two phenotypically distinct HIBM2 patients
    Conrad C Weihl
    Department of Neurology and Hope Center for Neurological Disorders, Washington University School of Medicine, 660 S Euclid Avenue, Saint Louis, MO 63110, USA
    Neuromuscul Disord 21:102-5. 2011
    ..His muscle biopsy showed prominent necrosis without rimmed vacuoles. This study expands the phenotype and illustrates the clinical spectrum of HIBM2 identified in a U.S. based neuromuscular clinic...
  6. pmc Sporadic inclusion body myositis: possible pathogenesis inferred from biomarkers
    Conrad C Weihl
    Department of Neurology and Hope Center for Neurological Disorders, Washington University School of Medicine, 660 S Euclid Avenue, Saint Louis, Missouri 63110, USA
    Curr Opin Neurol 23:482-8. 2010
    ..Our review will discuss current studies on these protein biomarkers and their utility in sIBM diagnosis...
  7. pmc Valosin-containing protein disease: inclusion body myopathy with Paget's disease of the bone and fronto-temporal dementia
    Conrad C Weihl
    Department of Neurology, Washington University School of Medicine, Saint Louis, MO 63110, USA
    Neuromuscul Disord 19:308-15. 2009
    ....
  8. pmc TDP-43 accumulation in inclusion body myopathy muscle suggests a common pathogenic mechanism with frontotemporal dementia
    C C Weihl
    Department of Neurology, Washington University School of Medicine, St Louis, Missouri 63110, USA
    J Neurol Neurosurg Psychiatry 79:1186-9. 2008
    ..This study adds sIBM and hereditary inclusion body myopathies to the growing list of TDP-43 positive inclusion diseases...
  9. ncbi request reprint Transgenic expression of inclusion body myopathy associated mutant p97/VCP causes weakness and ubiquitinated protein inclusions in mice
    Conrad C Weihl
    Department of Neurology, Washington University School of Medicine, 660 S Euclid Avenue, Saint Louis, MO 63110, USA
    Hum Mol Genet 16:919-28. 2007
    ..TgVCP-RH animals will be a valuable tool for understanding the pathogenesis of IBM and the role of the UPS in skeletal muscle...
  10. ncbi request reprint Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation
    Conrad C Weihl
    Department of Neurology, Washington University School of Medicine, 660 South Euclid Avenue, St Louis, MO 63110, USA
    Hum Mol Genet 15:189-99. 2006
    ..Undegraded mutant DeltaF508-CFTR also accumulates in these aggregates. We conclude that IBMPFD mutations in p97/VCP disrupt ERAD and that this may contribute to the pathogenesis of IBMPFD...

Research Grants5

  1. AAA ATPase p97/VCP and Inclusion Body Myopathy
    CONRAD WEIHL; Fiscal Year: 2007
    ..These studies will lend insight into the molecular and cellular mechanisms involved in IBM disease pathogenesis and are critical for identifying future therapeutic targets. ..
  2. IBMPFD MUTATIONS IMPAIR UPS FUNCTION
    CONRAD WEIHL; Fiscal Year: 2009
    ..We propose to explore the consequence of disease mutations in p97/VCP on UPS-mediated protein degradation in skeletal muscle and its relevance to aging related disorders such as inclusion body myositis and fronto-temporal dementia. ..
  3. IBMPFD MUTATIONS IMPAIR UPS FUNCTION
    Conrad C Weihl; Fiscal Year: 2010
    ..We propose to explore the consequence of disease mutations in p97/VCP on UPS-mediated protein degradation in skeletal muscle and its relevance to aging related disorders such as inclusion body myositis and fronto-temporal dementia. ..
  4. IBMPFD MUTATIONS IMPAIR UPS FUNCTION
    CONRAD WEIHL; Fiscal Year: 2009
    ..We propose to explore the consequence of disease mutations in p97/VCP on UPS-mediated protein degradation in skeletal muscle and its relevance to aging related disorders such as inclusion body myositis and fronto-temporal dementia. ..