The Administrative Core A will be responsible for the planning, development, coordination, and overall administration of the University of Massachusetts Medical School (UMMS) Wellstone MD CRC focused on Facioscapulohumeral Muscular Dystrophy (FSHD). Center leadership will continue under Dr. Charles Emerson, now at UMMS, and Dr. Louis Kunkel at Children's Boston, both highly experienced research administrators and accomplished scientists. The administrative unit for Core A will be located at UMMS, which has substantial resources, personnel and experience for management of a U54 Center. Core A will be the administrative hub to manage all Center resources and communications and carry out policies and procedures that establish and energized environment to enable effective communication and collaboration among all the Center constituents, including investigators, trainees, FSHD patients and advocacy groups, industry, NIH and the public. The Center will promote its mission through its website, which disseminates information on Center activities and research, and through its partnership with patient advocacy groups, particularly the FSH Society, which co-sponsors FSHD Research Workshops and Researcher-Patient Networking meetings to engage patients, researchers, trainees and NIH in our Center mission. Through Core A, the Center will promote the distribution of muscle cell lines from its Wellstone FSHD Cell Repository as a unique FSHD bio-resource. Internal committees will enable Center leadership, investigators and trainees to effectively utilize resources, organize internal dataflow and communications, and receive feedback on Center management, research progress and training. Regularly-scheduled Webex meetings will focus on data sharing and research planning, and an annual Wellstone Center Retreat at UMMS will bring investigators and trainees face-to-face with a Center Advisory Committee (CAC) and NIH program leaders to review research progress, set goals and align Center activities with the NIH Wellstone Center Network.
The Administrative Core A will provide administrative and communications support to enable the UMMS Wellstone Center to achieve its research mission to understand the underlying causes of Facioscapulohumeral Muscular Dystophy (FSHD), to develop therapeutics to treat this prevalent and debilitating disease, and to train research and clinical leaders in FSHD and muscular dystrophy research.
|Zhang, Yuanfan; King, Oliver D; Rahimov, Fedik et al. (2014) Human skeletal muscle xenograft as a new preclinical model for muscle disorders. Hum Mol Genet 23:3180-8|
|Mitsuhashi, Hiroaki; Mitsuhashi, Satomi; Lynn-Jones, Taylor et al. (2013) Expression of DUX4 in zebrafish development recapitulates facioscapulohumeral muscular dystrophy. Hum Mol Genet 22:568-77|
|Rahimov, Fedik; Kunkel, Louis M (2013) The cell biology of disease: cellular and molecular mechanisms underlying muscular dystrophy. J Cell Biol 201:499-510|
|Homma, Sachiko; Chen, Jennifer C J; Rahimov, Fedik et al. (2012) A unique library of myogenic cells from facioscapulohumeral muscular dystrophy subjects and unaffected relatives: family, disease and cell function. Eur J Hum Genet 20:404-10|
|Homma, Sachiko; Beermann, Mary Lou; Miller, Jeffrey Boone (2011) Peripheral nerve pathology, including aberrant Schwann cell differentiation, is ameliorated by doxycycline in a laminin-?2-deficient mouse model of congenital muscular dystrophy. Hum Mol Genet 20:2662-72|
|Reed, Patrick W; Bloch, Robert J (2011) Crystallin-gazing: unveiling enzymatic activity. J Neurochem 118:315-6|
|Roche, Joseph A; Ford-Speelman, Diana L; Ru, Lisa W et al. (2011) Physiological and histological changes in skeletal muscle following in vivo gene transfer by electroporation. Am J Physiol Cell Physiol 301:C1239-50|
|Rahimov, Fedik; King, Oliver D; Warsing, Leigh C et al. (2011) Gene expression profiling of skeletal muscles treated with a soluble activin type IIB receptor. Physiol Genomics 43:398-407|
|Arashiro, Patricia; Eisenberg, Iris; Kho, Alvin T et al. (2009) Transcriptional regulation differs in affected facioscapulohumeral muscular dystrophy patients compared to asymptomatic related carriers. Proc Natl Acad Sci U S A 106:6220-5|
|Vishnudas, Vivek K; Miller, Jeffrey Boone (2009) Ku70 regulates Bax-mediated pathogenesis in laminin-alpha2-deficient human muscle cells and mouse models of congenital muscular dystrophy. Hum Mol Genet 18:4467-77|