Search Results - "Colten, H R"

A mutation in the surfactant protein B gene responsible for fatal neonatal respiratory disease in multiple kindreds by NOGEE, L. M, GARNIER, G, DIETZ, H. C, SINGER, L, MURPHY, A. M, DEMELLO, D. E, COLTEN, H. R

“…To determine the molecular defect accounting for the deficiency of pulmonary surfactant protein B (SP-B) in full-term neonates who died from respiratory…”
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Lung transplantation for treatment of infants with surfactant protein B deficiency by Hamvas, Aaron, Nogee, Lawrence M., Mallory, George B., Spray, Thomas L., Huddleston, Charles B., August, Anna, Dehner, Louis P., deMello, Daphne E., Moxley, Michael, Nelson, Robert, Cole, F.Sessions, Colten, Harvey R.

“…Objective: To evaluate lung transplantation for treatment of surfactant protein B (SP-B) deficiency. Study design: We compared surfactant composition and…”
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Brief report : deficiency of pulmonary surfactant protein B in congenital alveolar proteinosis by NOGEE, L. M, DEMELLO, D. E, DEHNER, L. P, COLTEN, H. R

“…A lung obtained at autopsy from a 19-day-old full-term girl with meconium aspiration syndrome who had received mechanical ventilation with 100 percent oxygen…”
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Cachectin/tumor necrosis factor regulates hepatic acute-phase gene expression by PERLMUTTER, D. H, DINARELLO, C. A, PUNSAL, P. I, COLTEN, H. R

“…The monokine, cachectin/tumor necrosis factor (TNF) differs from interleukin 1 (IL-1) in primary structure and in recognition by a distinct cellular receptor…”
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Ultrastructure of lung in surfactant protein B deficiency by deMello, D E, Heyman, S, Phelps, D S, Hamvas, A, Nogee, L, Cole, S, Colten, H R

“…Congenital alveolar proteinosis (CAP), a cause of respiratory failure in fill-term newborns, often leads to death in infancy despite medical therapy. We…”
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Population-Based Estimates of Surfactant Protein B Deficiency by Cole, F. Sessions, Hamvas, Aaron, Rubinstein, Pablo, King, Erin, Trusgnich, Michelle, Nogee, Lawrence M, deMello, Daphne E, Colten, Harvey R

“…Surfactant protein B deficiency is a lethal cause of respiratory distress in infancy that results most commonly from a homozygous frameshift mutation…”
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Pretranslational modulation of acute phase hepatic protein synthesis by murine recombinant interleukin 1 (IL-1) and purified human IL-1 by RAMADORI, G, SIPE, J. D, DINARELLO, C. A, MIZEL, S. B, COLTEN, H. R

“…During the acute phase response to tissue injury or inflammation, the concentration of several plasma proteins change. Previous work (29-34) suggested a role…”
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Tissue-specific regulation of inflammation by Colten, H R

“…Proteins of the complement system are important effectors and modulators of inflammation. The complement cascade is triggered by microbes, tissue debris, and…”
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Polymorphism of the human complement C4 and steroid 21-hydroxylase genes: restriction fragment length polymorphisms revealing structural deletions, homoduplications, and size variants by SCHNEIDER, P. M, CARROLL, M. C, ALPER, C. A, RITTNER, C, WHITEHEAD, A. S, YUNIS, E. J, COLTEN, H. R

“…Several autoimmune disorders as well as congenital adrenal hyperplasia (CAH) are either associated or closely linked with genetic variants of the fourth…”
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Surfactant protein B deficiency: Antenatal diagnosis and prospective treatment with surfactant replacement by Hamvas, Aaron, Cole, F. Sessions, deMello, Daphne E., Moxley, Michael, Whitsett, Jeffrey A., Colten, Harvey R., Nogee, Lawrence M.

“…An infant with a family history of congenital alveolar proteinosis associated with surfactant protein B (SP-B) deficiency was identified when SP-B was not…”
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Local extrahepatic expression of complement genes C3, factor B, C2, and C4 is increased in murine lupus nephritis by PASSWELL, J, SCHREINER, G. F, NONAKA, M, BEUSCHER, H. U, COLTEN, H. R

“…Systemic lupus erythematosus (SLE) is associated with the presence of complement proteins and immune complexes in affected organs. Since complement proteins…”
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Genetic disruption of the murine complement C3 promoter region generates deficient mice with extrahepatic expression of C3 mRNA by Circolo, Antonella, Garnier, Gérard, Fukuda, Wataru, Wang, Xeufeng, Hidvegi, Tünde, Szalai, Alexander J, Briles, David E, Volanakis, John E, Wetsel, Rick A, Colten, Harvey R

“…Genetic deficiencies of the complement protein C3 occur naturally in humans and animal models and have been induced in mice by targeted deletion of the C3…”
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Expression and regulation of the murine serum amyloid A (SAA) gene in extrahepatic sites by RAMADORI, G, SIPE, J. D, COLTEN, H. R

“…Serum amyloid A (SAA) is an acute phase protein in several mammalian species. Its serum concentration is increased 100- to 1000-fold in acute and chronic…”
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gamma-Interferon increases expression of class III complement genes C2 and factor B in human monocytes and in murine fibroblasts transfected with human C2 and factor B genes by Strunk, R C, Cole, F S, Perlmutter, D H, Colten, H R

“…gamma-Interferon (IFN-gamma) is a well characterized lymphokine known to regulate many mononuclear phagocyte functions, including expression of class I and…”
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Plasma and cytoplasmic gelsolins are encoded by a single gene and contain a duplicated actin-binding domain by Kwiatkowski, David J, Stossel, Thomas P, Orkin, Stuart H, Mole, John E, Coltens, Harvey R, Yin, Helen L

“…Gelsolin is representative of a class of actin-modulating proteins found in lower eukaryotes to mammals, which sever actin filaments. Gelsolin found in the…”
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Transinhibition of C1 inhibitor synthesis in type I hereditary angioneurotic edema by KRAMER, J, ROSEN, F. S, COLTEN, H. R, RAJCZY, K, STRUNK, R. C

“…To ascertain the mechanism for decreased synthesis of C1 inhibitor (C1 INH) in certain patients with the autosomal dominant disorder hereditary angioneurotic…”
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Structure of a human serum amyloid A gene and modulation of its expression in transfected L cells by Woo, P, Sipe, J, Dinarello, C A, Colten, H R

“…The structure of a human serum amyloid A (SAA) genomic clone (SAAg9) has been analyzed and the nucleotide sequence of the coding regions is compared with that…”
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Type I human complement C2 deficiency. A 28-base pair gene deletion causes skipping of exon 6 during RNA splicing by C A Johnson, P Densen, R K Hurford, Jr, H R Colten, R A Wetsel

“…Two variants of a genetic deficiency of complement protein C2 (C2D) have been previously identified. No C2 protein translation is detected in type I…”
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Murine complement C2 and factor B genomic and cDNA cloning reveals different mechanisms for multiple transcripts of C2 and B by Ishikawa, N, Nonaka, M, Wetsel, R.A., Colten, H.R.

“…Murine genomic and cDNA clones were isolated to ascertain the mechanisms accounting for previously recognized multiple forms of complement C2 and factor B mRNA…”
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Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis by Auerbach, H S, Williams, M, Kirkpatrick, J A, Colten, H R

“…A randomised, double-blind, placebo-controlled study examined the effects of alternate-day prednisone therapy on morbidity and progression of lung disease in…”
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