title: Circulating progenitor cells and scleroderma.
abstract: Scleroderma (systemic sclerosis) is a disease of unknown origins that involves tissue ischemia and fibrosis in the skin and internal organs such as the lungs. The tissue ischemia is due to a lack of functional blood vessels and an inability to form new blood vessels. Bone marrow--derived circulating endothelial progenitor cells play a key role in blood vessel repair and neovascularization. Scleroderma patients appear to have defects in the number and function of circulating endothelial progenitor cells. Scleroderma patients also develop fibrotic lesions, possibly as the result of tissue ischemia. Fibroblast-like cells called fibrocytes that differentiate from a different pool of bone marrow-derived circulating progenitor cells seem to be involved in this process. Manipulating the production, function, and differentiation of circulating progenitor cells represents an exciting new possibility for treating scleroderma.
authors.affiliation: Department of Biochemistry and Cell Biology, MS-140, Rice University, 6100 South Main Street, Houston, TX 77005, USA. richard@rice.edu
authors.forename: Richard H
authors.initials: RH
authors.lastname: Gomer
mesh_terms: D003520:Cyclophosphamide / Q000627:therapeutic use; D004730:Endothelium, Vascular / Q000502:physiology*; D005347:Fibroblasts / Q000502:physiology*; D006412:Hematopoietic Stem Cells / Q000502:physiology*; D006801:Humans; D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D012595:Scleroderma, Systemic / Q000188:drug therapy / Q000503:physiopathology*; D033581:Stem Cell Transplantation; D013234:Stem Cells
chemical_list: D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide