Research Topics
| Francis McCormackSummaryAffiliation: University of Cincinnati Country: USA Publications
Research Grants
| Collaborators
|
Detail Information
Publications
The carbohydrate recognition domain of surfactant protein A mediates binding to the major surface glycoprotein of Pneumocystis cariniiF X McCormack
Department of Internal Medicine, University of Cincinnati College of Medicine, Cincinnati, Ohio 45267, USA
Biochemistry 36:8092-9. 1997..Deletion of the N-linked oligosaccharides from SP-A by mutagenesis of the consensus sequences for glycosylation had no effect on binding. We conclude that the CRD mediates the binding of SP-A to oligosaccharides attached to MSG...- Macrophage-independent fungicidal action of the pulmonary collectinsFrancis X McCormack
Veterans Affairs Medical Center, Cincinnati, Ohio 45220, USA
J Biol Chem 278:36250-6. 2003.... - New concepts in collectin-mediated host defense at the air-liquid interface of the lungFrancis X McCormack
Division of Pulmonary and Critical Care Medicine, University of Cincinnati, Cincinnati, OH 45267 0564, USA
Respirology 11:S7-10. 2006..Recent studies suggest that these proteins have a direct effect on the growth and viability of Gram-negative bacteria, Mycoplasma pneumoniae and Histoplasma capsulatum... 
Efficacy and safety of sirolimus in lymphangioleiomyomatosisFrancis X McCormack
University of Cincinnati, Cincinnati, OH, USA
N Engl J Med 364:1595-606. 2011..Sirolimus (also called rapamycin) inhibits mTOR and has shown promise in phase 1-2 trials involving patients with LAM...- The pulmonary collectins, SP-A and SP-D, orchestrate innate immunity in the lungFrancis X McCormack
Department of Medicine, Division of Pulmonary and Critical Care Medicine, Children's Hospital Medical Center, University of Cincinnati, Cincinnati, OH 45267-0564, USA
J Clin Invest 109:707-12. 2002 - Pulmonary cysts consistent with lymphangioleiomyomatosis are common in women with tuberous sclerosis: genetic and radiographic analysisFrancis McCormack
Department of Pediatrics, Division of Pulmonary and Critical Care Medicine, University of Cincinnati, Cincinnati, OH 45267-0564, USA
Chest 121:61S. 2002 
Lymphangioleiomyomatosis: a clinical updateFrancis X McCormack
Division of Pulmonary, Critical Care and Sleep Medicine, University of Cincinnati, MSB 6053, 231 Albert Sabin Way, Cincinnati, OH 45267 0564, USA
Chest 133:507-16. 2008..Multicenter clinical trials based on several well-defined molecular targets are currently underway in the United States and Europe...- Alanine mutagenesis of surfactant protein A reveals that lipid binding and pH-dependent liposome aggregation are mediated by the carbohydrate recognition domainF X McCormack
Division of Pulmonary Critical Care Medicine, University of Cincinnati College of Medicine, Ohio, USA
Biochemistry 36:13963-71. 1997.... - Structure, processing and properties of surfactant protein AF X McCormack
Division of Pulmonary and Critical Care Medicine, University of Cincinnati College of Medicine, 231 Bethesda Ave, Cincinnati, OH 45267 0564, USA
Biochim Biophys Acta 1408:109-31. 1998..Recently developed genetically manipulated animal models are beginning to clarify the critical physiological roles for SP-A in the normal lung, and in the pathophysiology of pulmonary disease... - Deletion mapping of N-terminal domains of surfactant protein A. The N-terminal segment is required for phospholipid aggregation and specific inhibition of surfactant secretionF X McCormack
Division of Pulmonary Critical Care Medicine, Department of Internal Medicine, University of Cincinnati College of Medicine, Cincinnati, Ohio 45267, USA
J Biol Chem 274:3173-81. 1999.... - The collagen-like region of surfactant protein A (SP-A) is required for correction of surfactant structural and functional defects in the SP-A null mouseM Ikegami
Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of Cincinnati School of Medicine, Cincinnati, Ohio 45229 3039, USA
J Biol Chem 276:38542-8. 2001.... - Immunosuppressed surfactant protein A-deficient mice have increased susceptibility to Pneumocystis carinii infectionM J Linke
Research Service, Department of Veterans Affairs Medical Center, and Divisions of Infectious Diseases and Pulmonary Critical Care Medicine, University of Cincinnati, Cincinnati, OH 45220, USA
J Infect Dis 183:943-52. 2001..carinii to the macrophages. Thus, SP-A plays a role in host defense against P. carinii in vivo, perhaps by functioning as a nonimmune opsonin... - Three-dimensional structure of rat surfactant protein A trimers in association with phospholipid monolayersN Palaniyar
Department of Internal Medicine, University of Cincinnati, Ohio 45267 0564, USA
Biochemistry 39:6310-6. 2000.... - Functional mapping of surfactant protein AF X McCormack
Division of Pulmonary and Critical Case Medicine, Univ of Cincinnati College of Medicine, 231 Albert Sabin Way, Cincinnati, OH 45267 0564, USA
Pediatr Pathol Mol Med 20:293-318. 2001..My review focuses on the putative biological functions of SP-A that are supported by experiments in mammals and on the structural basis of SP-A function... - Surfactant lipid peroxidation damages surfactant protein A and inhibits interactions with phospholipid vesiclesA I Kuzmenko
Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Cincinnati School of Medicine, Cincinnati, OH 45267, USA
J Lipid Res 45:1061-8. 2004..We conclude that exposure of SP-A to LPO results in oxidative modification and functional inactivation of SP-A by phospholipid radicals... - Mutational and radiographic analysis of pulmonary disease consistent with lymphangioleiomyomatosis and micronodular pneumocyte hyperplasia in women with tuberous sclerosisD N Franz
Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA
Am J Respir Crit Care Med 164:661-8. 2001..This prospective analysis demonstrates that cystic and nodular pulmonary changes consistent with LAM and MMPH are common in women with TSC... - Patient perspectives on management of pneumothorax in lymphangioleiomyomatosisLisa R Young
Department of Medicine, University of Cincinnati, Division of Pulmonary and Critical Care, Cincinnati, OH 45267 0564, USA
Chest 129:1267-73. 2006..Study design and participants: A 23-item questionnaire evaluating opinions of pneumothorax treatment was distributed to 615 patients in the LAM Foundation patient database, with a response rate of 52%... - Lung-restricted macrophage activation in the pearl mouse model of Hermansky-Pudlak syndromeLisa R Young
Department of Medicine, Division of Pulmonary and Critical Care, University of Cincinnati, OH 45267, USA
J Immunol 176:4361-8. 2006..Similar abnormalities were identified in AMs and BAL from another HPS model, pale ear HPS1 mice. We conclude that the lungs of HPS mice exhibit hyperresponsiveness to LPS and constitutive and organ-specific macrophage activation... - Neither SP-A nor NH2-terminal domains of SP-A can substitute for SP-D in regulation of alveolar homeostasisLiqian Zhang
Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center, OH 45229, USA
Am J Physiol Lung Cell Mol Physiol 291:L181-90. 2006.... - Pulmonary collectins selectively permeabilize model bacterial membranes containing rough lipopolysaccharideAlexander I Kuzmenko
Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Cincinnati, 231 Albert Sabin Way, Cincinnati, Ohio 45267 0564, USA
Biochemistry 45:2679-85. 2006..Incorporation of cholesterol into the membrane enhances resistance to permeabilization by SP-A, most likely by increasing the packing density and membrane rigidity... - Pleural disease in lymphangioleiomyomatosisKhalid F Almoosa
Department of Internal Medicine, Division of Pulmonary, Critical Care, and Sleep Medicine, University of Cincinnati College of Medicine, Cincinnati, OH 45267 0564, USA
Clin Chest Med 27:355-68. 2006..Because of the rarity of the LAM, few data exist on whether the occurrence of pleural complications in LAM accelerates functional decline or portends a worse prognosis... - Correction of pulmonary abnormalities in Sftpd-/- mice requires the collagenous domain of surfactant protein DPaul S Kingma
Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
J Biol Chem 281:24496-505. 2006..The collagen domain of SP-D is required for the regulation of pulmonary macrophage activation, airspace remodeling, and surfactant lipid homeostasis... - Air travel in women with lymphangioleiomyomatosisStacey Pollock-Barziv
Women s College Research Institute, Women s College Hospital, Toronto, Ontario, Canada
Thorax 62:176-80. 2007..A questionnaire-based evaluation of air travel in patients with LAM was conducted to determine experiences aboard commercial aircraft... - Molecular pathogenesis of lymphangioleiomyomatosis: lessons learned from orphansStephen C Juvet
National Jewish Medical and Research Center, 1400 Jackson Street, Denver, CO 80206, USA
Am J Respir Cell Mol Biol 36:398-408. 2007..This knowledge served as the foundation for novel therapeutic approaches that are currently being used in human clinical trials... - Management of pneumothorax in lymphangioleiomyomatosis: effects on recurrence and lung transplantation complicationsKhalid F Almoosa
Division of Pulmonary and Critical Care, University of Cincinnati, Cincinnati, OH 45267 0564, USA
Chest 129:1274-81. 2006..Chemical or surgical pleurodesis are often required to prevent recurrence. However, their efficacy in LAM is unclear, and whether they contribute to perioperative complications during lung transplantation is uncertain... - The resident scholar program: a research training opportunity for internal medicine house staffAbigail B Byrnes
University of Cincinnati, Cincinnati, Ohio, USA
J Cancer Educ 22:47-9. 2007..The Resident Scholar Program (RSP) at the University of Cincinnati, Department of Internal Medicine was created to overcome these challenges... - Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosisJohn J Bissler
Division of Nephrology and Hypertension, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229 3039, USA
N Engl J Med 358:140-51. 2008..The drug sirolimus suppresses mTOR signaling... - Diagnostic potential of serum VEGF-D for lymphangioleiomyomatosisLisa R Young
N Engl J Med 358:199-200. 2008 - Surfactant proteins A and D inhibit the growth of Gram-negative bacteria by increasing membrane permeabilityHuixing Wu
Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Cincinnati, Cincinnati, Ohio, USA
J Clin Invest 111:1589-602. 2003.... - Pulmonary surfactant protein A up-regulates activity of the mannose receptor, a pattern recognition receptor expressed on human macrophagesAlison A Beharka
Veterans Affairs Medical Center and Division of Infectious Diseases, Department of Medicine and Microbiology, Interdisciplinary Immunology Program, University of Iowa, Iowa City, IA 52242, USA
J Immunol 169:3565-73. 2002..SP-A contributes to the alternative activation state of the AM in the lung... - Recurrent lymphangiomyomatosis after transplantation: genetic analyses reveal a metastatic mechanismMagdalena Karbowniczek
Fox Chase Cancer Center, 7701 Burholme Avenue, Philadelphia, PA 19111, USA
Am J Respir Crit Care Med 167:976-82. 2003..In addition, the patient had no evidence of a renal angiomyolipoma at autopsy and therefore demonstrated for the first time that somatic TSC2 mutations cause LAM in patients without angiomyolipomas... - Surfactant protein A inhibits lipopolysaccharide-induced in vivo production of interleukin-10 by mononuclear phagocytes during lung inflammationSophie Chabot
Unité de Défense Innée et Inflammation, Unité Associée Institut Pasteur Institut National de la Santé et de la Recherche Médicale, U485, Paris, France
Am J Respir Cell Mol Biol 28:347-53. 2003..In summary, this study demonstrates for the first time an in vivo inhibitory role of SP-A on the anti-inflammatory activity of MP, through inhibition of IL-10 production... - Pulmonary surfactant protein-A (SP-A) restores the surface properties of surfactant after oxidation by a mechanism that requires the Cys6 interchain disulfide bond and the phospholipid binding domainKarina Rodriguez Capote
Departments of Obstetrics Gynecology and Biochemistry, Canadian Institutes of Health Research Group in Fetal and Neonatal Health and Development, University of Western Ontario, London N6A 5A5, Canada
J Biol Chem 278:20461-74. 2003.... - Crystal structure of trimeric carbohydrate recognition and neck domains of surfactant protein AJames F Head
Department of Physiology and Biophysics, Boston University School of Medicine, Boston, Massachusetts 02118, USA
J Biol Chem 278:43254-60. 2003.... - Cutting edge: the immunostimulatory activity of the lung surfactant protein-A involves Toll-like receptor 4Loic Guillot
Unité de Défense Innée et Inflammation, Institut Pasteur, Institut National de la Sante et de la Recherche Medicale, Unité 485, Paris, France
J Immunol 168:5989-92. 2002..These findings support the concept that TLR4 is a pattern recognition receptor that signals in response to both foreign pathogens and endogenous host mediators... - The role of pulmonary collectin N-terminal domains in surfactant structure, function, and homeostasis in vivoNades Palaniyar
Division of Pulmonary Critical Care Medicine, Department of Medicine, Children s Hospital Research Foundation, University of Cincinnati School of Medicine, Cincinnati, OH 45267 0564, USA
J Biol Chem 277:26971-9. 2002.... - Bordetella pertussis lipopolysaccharide resists the bactericidal effects of pulmonary surfactant protein ALyndsay M Schaeffer
Department of Molecular Genetics, Biochemistry, and Microbiology, University of Cincinnati, Cincinnati, OH 45267, USA
J Immunol 173:1959-65. 2004..pertussis toxin that has been shown to depress neutrophil activity. We conclude that the LPS of wild-type B. pertussis shields the bacteria from SP-A-mediated clearance, possibly by sterically limiting access to the lipid A region... - Interactions of pulmonary collectins with Bordetella bronchiseptica and Bordetella pertussis lipopolysaccharide elucidate the structural basis of their antimicrobial activitiesLyndsay M Schaeffer
Department of Molecular Genetics, Biochemistry, and Microbiology, University of Cincinnati, 231 Albert Sabin Way, ML 0524, Cincinnati, OH 45267, USA
Infect Immun 72:7124-30. 2004..We conclude that the terminal trisaccharide protects Bordetella species from the bactericidal functions of SP-A and SP-D. The O antigen and palmitoylated lipid A of B. bronchiseptica play no role in this resistance... - Surfactant protein A is a principal and oxidation-sensitive microbial permeabilizing factor in the alveolar lining fluidAlexander I Kuzmenko
Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, University of Cincinnati School of Medicine, Cincinnati, Ohio 45267 0564, USA
J Biol Chem 280:25913-9. 2005.... - Pulmonary surfactant protein A activates a phosphatidylinositol 3-kinase/calcium signal transduction pathway in human macrophages: participation in the up-regulation of mannose receptor activityAlison A Beharka
Interdisciplinary Program in Immunology and Department of Internal Medicine, University of Iowa, Iowa City, IA 52240, USA
J Immunol 175:2227-36. 2005..These studies provide further evidence that SP-A produced in the lung plays a role in modulating macrophage biology, thereby contributing to the alternative activation state of the alveolar macrophage... - Pulmonary surfactant and inflammation in septic adult mice: role of surfactant protein AJaret L Malloy
Department of Physiology, Lawson Health Research Institute, University of Western Ontario, London, Ontario, Canada N6A 4V2
J Appl Physiol 92:809-16. 2002..In addition, we did not detect a role for SP-A in regulating surfactant phospholipid pool sizes or pulmonary inflammation in septic mice... - S-LAM in a man?Francis X McCormack
Am J Respir Crit Care Med 176:3-5. 2007 - Antimicrobial activity of native and synthetic surfactant protein B peptidesMarnie A Ryan
Division of Pulmonary Biology, Cincinnati Children s Hospital Medical Center, and University of Cincinnati College of Medicine, OH 45229 3039, USA
J Immunol 176:416-25. 2006..These results suggest that the role of endogenous SP-B in host defense may be limited; however, synthetic peptides derived from SP-B may be useful in the treatment of bacterial pneumonias... - LymphangioleiomyomatosisFrancis X McCormack
Division of Pulmonary and Critical Care, The University of Cincinnati School of Medicine, Cincinnati, Ohio, USA
MedGenMed 8:15. 2006 - Lymphangioleiomyomatosis: insights about, and from, a rare diseaseKathy E Sietsema
Am J Respir Crit Care Med 168:1405-6. 2003 - Pulmonary surfactant protein a inhibits macrophage reactive oxygen intermediate production in response to stimuli by reducing NADPH oxidase activityJoy E Crowther
Interdisciplinary Graduate Program in Immunology, University of Iowa, Iowa City, IA 52240, USA
J Immunol 172:6866-74. 2004..These data support an anti-inflammatory role for SP-A in pulmonary homeostasis by inhibiting Mphi production of ROI through a reduction in NADPH oxidase activity... - The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollmentJay H Ryu
Division of Pulmonary and Critical Care Medicine, Desk East 18, Mayo Clinic, 200 1st Street SW, Rochester, MN 55905, USA
Am J Respir Crit Care Med 173:105-11. 2006..Previous descriptions of clinical characteristics of subjects with lymphangioleiomyomatosis have been based on a limited number of patients... - Susceptibility of Hermansky-Pudlak mice to bleomycin-induced type II cell apoptosis and fibrosisLisa R Young
University of Cincinnati, Pulmonary, Critical Care, and Sleep Medicine, 231 Albert Sabin Way, Cincinnati, OH 45267, USA
Am J Respir Cell Mol Biol 37:67-74. 2007..We conclude that the trafficking abnormalities in HPS promote alveolar apoptosis and pulmonary fibrosis in response to bleomycin challenge...
Research Grants
- The 2007 LAM Foundation Lymphangioleiomyomatosis International Research ConferencFrancis McCormack; Fiscal Year: 2007....
- PHASE III TRIAL FO SIROLIMUS IN LYMPHANGIOLEIOMYLMATOSISFrancis McCormack; Fiscal Year: 2007..Patient safety will be monitored by an NIH Data Safety Monitoring Board (DSMB). The MILES trial is designed as a pivotal study to determine if sirolimus is effective in stabilizing or improving lung function in patients with LAM. ..
- Lymphangioleiomyomatosis Research Conference 2006Francis McCormack; Fiscal Year: 2006....
- 2005 LAM Foundation Lymphangioleiomyomatosis ResearchFrancis McCormack; Fiscal Year: 2005..abstract_text> ..
- Novel Antioxidant Mechanisms of Surfactant ProteinsFrancis McCormack; Fiscal Year: 2006..The results will provide important insights into the roles of the surfactant proteins in preventing oxidative lung disease and may be useful for the design of novel recombinant proteins and peptides of therapeutic value. ..
- Lymphangioleimyomatosis Research ConferenceFrancis McCormack; Fiscal Year: 2004..abstract_text> ..
- Lymphangioleiomyomatosis Research ProjectFrancis McCormack; Fiscal Year: 2003..abstract_text> ..
- Lymphangioleiomyomatosis Research ProjectFrancis McCormack; Fiscal Year: 2002..Efforts have been made to attract established investigators from related disciplines who can complement the expertise of the LAM investigators. ..
- SURFACTANT PROTEIN A IN PULMONARY HOST DEFENSEFrancis McCormack; Fiscal Year: 2002..These studies will elucidate the role of SP-A in the innate immune defense of the lung, and may assist in the design of optimal antimicrobial strategies. ..
- PHASE III TRIAL FO SIROLIMUS IN LYMPHANGIOLEIOMYLMATOSISFrancis McCormack; Fiscal Year: 2009..Patient safety will be monitored by an NIH Data Safety Monitoring Board (DSMB). The MILES trial is designed as a pivotal study to determine if sirolimus is effective in stabilizing or improving lung function in patients with LAM. ..