the homozygote vcpr155hr155h mouse model exhibits accelerated human vcp-associated disease pathology纯合体vcpr155hr155h鼠标模型展览加速人类vcp-associated疾病病理学.pdfVIP

The Homozygote VCPR155H/R155H Mouse Model Exhibits Accelerated Human VCP-Associated Disease Pathology ` 1 1 2 3 1 Angele Nalbandian , Katrina J. Llewellyn , Masashi Kitazawa , Hong Z. Yin , Mallikarjun Badadani , 4 5 1 6 3,7 Negar Khanlou , Robert Edwards , Christopher Nguyen , Jogeshwar Mukherjee , Tahseen Mozaffar , 8 3 1 Giles Watts , John Weiss , Virginia E. Kimonis * 1 Department of Pediatrics, University of California Irvine, Irvine, California, United States of America, 2 Department of Molecular and Cell Biology, University of California Merced, Merced, California, United States of America, 3 Department of Neurology, Anatomy and Neurobiology, University of California Irvine, Irvine, California, United States of America, 4 Department of Pathology and Lab Medicine, University of California Los Angeles, Los Angeles, California, United States of America, 5 Department of Pathology, University of California Irvine, Irvine, California, United States of America, 6 Department of Radiological Sciences, University of California Irvine, Irvine, California, United States of America, 7 Department of Orthopedics, University of California Irvine, Irvine, California, United States of America, 8 Department of Cell Biology and Biochemistry, University of East Anglia, Norwich, Norfolk, United Kingdom Abstract Valosin containing protein (VCP) mutations are the cause of hereditary inclusion body myopathy, Paget’s disease of bone, frontotemporal dementia (IBMPFD). VCP