Browsing by Author "Williams, David"
Now showing items 1-8 of 8
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Contributions of the RhoGEF activity of p210 BCR/ABL to disease progression
Tala, Ilona; Chen, Ru; Hu, Tinghui; Fitzpatrick, Ethan R; Williams, David A; Whitehead, Ian P (2014)We have previously identified a tyrosine kinase-independent, guanine nucleotide exchange factor (GEF) activity that is contained within the region of p210 BCR/ABL that distinguishes it from p190 BCR/ABL. In the current ... -
Gaining the hard yard: pre-clinical evaluation of lentiviral-mediated gene therapy for the treatment of β-thalassemia
Milsom, Michael D; Williams, David Allan (WILEY-VCH Verlag, 2010)Gene therapy is one potential novel therapeutic avenue for the treatment of inherited monogenic disorders. Diseases of the blood are frequent targets for gene therapy because it is relatively easy to harvest haematopoietic ... -
Guanine Nucleotide Exchange Factor Vav1 Regulates Perivascular Homing and Bone Marrow Retention of Hematopoietic Stem and Progenitor Cells
Sanchez-Aguilera, Abel; Lee, Yun-Jung; Lo Celso, Cristina; Ferraro, Francesca; Brumme, Kristina; Mondal, Subhanjan; Kim, Chaekyun; Dorrance, Adrienne; Luo, Hongbo; Scadden, David Thomas; Williams, David Allan (Proceedings of the National Academy of Sciences, 2011)Engraftment and maintenance of hematopoietic stem and progenitor cells (HSPC) depend on their ability to respond to extracellular signals from the bone marrow microenvironment, but the critical intracellular pathways ... -
Leukemic survival factor SALL4 contributes to defective DNA damage repair
Wang, Fei; Gao, Chong; Lu, Jiayun; Tatetsu, Hiro; Williams, David A.; Müller, Lars U; Cui, Wei; Chai, Li (2016)SALL4 is aberrantly expressed in human myelodysplastic syndromes (MDS) and acute myeloid leukemia (AML). We have generated a SALL4 transgenic (SALL4B Tg) mouse model with pre-leukemic MDS-like symptoms that transform to ... -
Loss of Function of TET2 Cooperates with Constitutively Active KIT in Murine and Human Models of Mastocytosis
De Vita, Serena; Schneider, Rebekka K.; Garcia, Michael; Wood, Jenna; Gavillet, Mathilde; Ebert, Benjamin L.; Gerbaulet, Alexander; Roers, Axel; Levine, Ross L.; Mullally, Ann; Williams, David A. (Public Library of Science, 2014)Systemic Mastocytosis (SM) is a clonal disease characterized by abnormal accumulation of mast cells in multiple organs. Clinical presentations of the disease vary widely from indolent to aggressive forms, and to the ... -
Mathematical Modeling of Erythrocyte Chimerism Informs Genetic Intervention Strategies for Sickle Cell Disease
Brendel, Christian; Renella, Raffaele; Michor, Franziska; Altrock, Philipp; Orkin, Stuart; Williams, David (Wiley, 2016-07-14)Recent advances in gene therapy and genome-engineering technologies offer the opportunity to correct sickle cell disease (SCD), a heritable disorder caused by a point mutation in the beta-globin gene. The developmental ... -
PTEN Negatively Regulates Engulfment of Apoptotic Cells by Modulating Activation of Rac GTPase
Mondal, Subhanjan; Ghosh-Roy, Saurabh; Loison, Fabien; Li, Yitang; Jia, Yonghui; Harris, Chad; Williams, David Allan; Luo, Hongbo (American Association of Immunologists, 2011)Efficient clearance of apoptotic cells by phagocytes (efferocytosis) is critical for normal tissue homeostasis and regulation of the immune system. Apoptotic cells are recognized by a vast repertoire of receptors on ... -
Rac GTPases in Human Diseases
Pai, Sung-Yun; Kim, Chaekyun; Williams, David A. (IOS Press, 2010)Rho GTPases are members of the Ras superfamily of GTPases that regulate a wide variety of cellular functions. While Rho GTPase pathways have been implicated in various pathological conditions in humans, to date coding ...