title: Deficient expression of bactericidal/permeability-increasing protein in immunocompromised hosts: translational potential of replacement therapy.
abstract: BPI (bactericidal/permeability-increasing protein) is a 55 kDa anti-infective molecule expressed in neutrophil and eosinophil granules and on some epithelial cells. BPI's high affinity for the lipid A region of endotoxin targets its opsonizing, microbicidal and endotoxin-neutralizing activities towards Gram-negative bacteria. Several immunocompromised patient populations demonstrate BPI deficiency, including newborns, those with anti-neutrophil cytoplasmic antibodies (as in cystic fibrosis and HIV infection) and those exposed to radiochemotherapy. BPI may be replenished by administering agents that induce its expression or by administration of recombinant BPI congeners, potentially shielding BPI-deficient individuals against Gram-negative bacterial infection, endotoxemia and its toxic sequelae.
authors.affiliation: Children's Hospital Boston, Harvard Medical School, Boston, MA 02115, USA.
authors.forename: Christine D
authors.initials: CD
authors.lastname: Palmer
authors.forename: Eva C
authors.initials: EC
authors.lastname: Guinan
authors.forename: Ofer
authors.initials: O
authors.lastname: Levy
mesh_terms: D000818:Animals; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D023181:Antimicrobial Cationic Peptides / Q000172:deficiency* / Q000378:metabolism; D001798:Blood Proteins / Q000172:deficiency* / Q000378:metabolism; D019446:Endotoxemia / Q000517:prevention & control; D004847:Epithelial Cells / Q000378:metabolism; D016905:Gram-Negative Bacterial Infections / Q000188:drug therapy / Q000276:immunology*; D006801:Humans; D007113:Immunity, Innate; D016867:Immunocompromised Host; D007962:Leukocytes / Q000378:metabolism; D044045:Lipoxins / Q000627:therapeutic use; D058990:Molecular Targeted Therapy
chemical_list: D000900:Anti-Bacterial Agents; D023181:Antimicrobial Cationic Peptides; D001798:Blood Proteins; D044045:Lipoxins; C021623:bactericidal permeability increasing protein