Chris J. Lees

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Recessive dystrophic epidermolysis bullosa (RDEB) is an inherited blistering skin disorder caused by mutations in the COL7A1 gene-encoding type VII collagen (Col7), the major component of anchoring fibrils at the dermal-epidermal junction. Individuals with RDEB develop painful blisters and mucosal erosions, and currently, there are no effective forms of(More)
We have developed a mouse system by which to track the migration and homing of cells in a setting of bone marrow transplantation (BMT)-induced graft-versus-host disease (GVHD) after systemic infusion using enhanced green fluorescence protein (eGFP) transgenic (Tg) cells and a simple application of a fluorescence stereomicroscope outfitted with a color(More)
Mucopolysaccharidosis type I (MPS IH; Hurler syndrome) is a congenital deficiency of α-L-iduronidase, leading to lysosomal storage of glycosaminoglycans that is ultimately fatal following an insidious onset after birth. Hematopoietic cell transplantation (HCT) is a life-saving measure in MPS IH. However, because a suitable hematopoietic donor is not found(More)
The skin biopsy was cut into 2 × 2 mm pieces. The dermis was peeled off and trapped under a sterile cover slip. Human fibroblast media consisted of DMEM (Invitrogen, Carlsbad, CA), 10% fetal bovine serum (Invitrogen), and 1% penicillin/streptomycin (Invitrogen). Medium was changed every 2 days until the culture became 90% confluent, at which point it was(More)
We have developed a mouse system by which to track the migration and homing of cells in a setting of bone marrow transplantation (BMT)–induced graft-versushost disease (GVHD) after systemic infusion using enhanced green fluorescence protein (eGFP) transgenic (Tg) cells and a simple application of a fluorescence stereomicroscope outfitted with a color(More)
Spontaneous reversion of disease-causing mutations has been observed in some genetic disorders. In our clinical observations of severe generalized recessive dystrophic epidermolysis bullosa (RDEB), a currently incurable blistering genodermatosis caused by loss-of-function mutations in COL7A1 that results in a deficit of type VII collagen (C7), we have(More)
Otitis media has a complex multifactorial pathogenesis, and the middle ear inflammatory response is typified by the accumulation of cellular and chemical mediators in middle ear effusion. However, specific biochemical and immunochemical factors that may be responsible for the severity or chronicity of otitis media have not been identified. Identification of(More)
OBJECTIVE To investigate whether syngeneic BM-derived DCs generated in vitro and fused with syngeneic C1498 tumor cells (murine AML line) could induce a better antitumor protective effect compared to similarly generated DCs pulsed with C1498 lysate with or without co-injection of a class B CpG oligodeoxynucleotide (CpG 7909) in vivo. METHODS DCs were(More)
Platelet-activating factor (PAF) is a naturally occurring phospholipid that acts as a pleiotropic mediator of inflammation via specific membrane receptors. It has been demonstrated in recent years that PAF is a strong secretagogue of mucous glycoprotein (MGP) in airways and middle ear epithelium. MGP secretion accompanies otitis media with effusion (OME)(More)
1Division of Hematology-Oncology, Blood and Marrow Transplantation, Department of Pediatrics, University of MN, Minneapolis, MN, and 2Stem Cell Transplantation Program, Division of Pediatric Hematology Oncology, Manton Center for Orphan Disease Research, Children’s Hospital Boston, and Dana-Farber Cancer Institute; Department of Biological Chemistry and(More)
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