Abnormal expression of surfactant protein C and lung disease

American Journal of Respiratory Cell and Molecular Biology

Volumn 26, Issue 6, 2002, Pages 641-644

  Nogee, Lawrence M ^a  

^a JOHNS HOPKINS HOSPITAL   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

LUNG SURFACTANT; PROTEOLIPID;

ANIMAL; ARTICLE; HUMAN; LUNG DISEASE; METABOLISM; PATHOPHYSIOLOGY;

ANIMALS; HUMANS; LUNG DISEASES; PROTEOLIPIDS; PULMONARY SURFACTANTS;

EID: 0036014820     PISSN: 10441549     EISSN: None     Source Type: Journal    
DOI: 10.1165/ajrcmb.26.6.f241     Document Type: Review

References (45)

1. Hyaline membrane disease
2. Pulmonary surfactant therapy
3. Surfactant for acute respiratory distress syndrome
4. Hydrophobic surfactant-associated protein in whole lung surfactant and its importance for biophysical activity in lung surfactant extracts used for replacement therapy
5. Function of surfactant proteins B and C
6. Biophysical and biological activity of a synthetic 8.7-kDa hydrophobic pulmonary surfactant protein SP-B
7. Comparison of functional efficacy of surfactant protein B analogues in lavaged rats
8. Lung function in premature lambs and rabbits treated with a recombinant SP-C surfactant
9. Importance of hydrophobic apoproteins as constituents of clinical exogenous surfactants
10. The ontogeny and distribution of surfactant protein B in human fetuses and newborns
11. Glucocorticoid enhances surfactant proteolipid Phe and pVal synthesis and RNA in fetal lung
12. Temporal-spatial distribution of SP-B and SP-C proteins and mRNAs in developing respiratory epithelium of human lung
13. Increased expression of pulmonary surfactant proteins in oxygen-exposed rats
14. Synthesis, processing and secretion of surfactant proteins B and C
15. Genetic element from human surfactant protein SP-C gene confers bronchiolar-alveolar cell specificity in transgenic mice
16. Transgenic models for study of lung morphogenesis and repair: Parker B. Francis lecture
17. Two SP-C genes encoding human pulmonary surfactant proteolipid
18. Genetics of the hydrophobic surfactant proteins
19. Low molecular weight human pulmonary surfactant protein (SP5): Isolation, characterization, and cDNA and amino acid sequences
20. The pulmonary surfactant protein C (SP-C) precursor is a type 11 transmembrane protein
21. Secretion of surfactant protein C, an integral membrane protein, requires the N-terminal propeptide
22. Post-translational processing of surfactant protein-C proprotein: Targeting motifs in the NH(2)-terminal flanking domain are cleaved in late compartments
23. Hydrophobic surfactant-associated polypeptides: SP-C is a lipopeptide with two palmitoylated cysteine residues, whereas SP-B lacks covalently linked fatty acyl groups
24. Surfactant protein C precursor is palmitoylated and associates with subcellular membranes
25. Structure and properties of surfactant protein C
26. Targeted disruption of the surfactant protein B gene disrupts surfactant homeostasis, causing respiratory failure in newborn mice
27. A mutation in the surfactant protein B gene responsible for fatal neonatal respiratory disease in multiple kindreds
28. Surfactant protein-B deficiency
29. Aberrant processing of surfactant protein C in hereditary SP-B deficiency
30. Allelic heterogeneity in hereditary surfactant protein B (SP-B) deficiency
31. Lamellar body formation in normal and surfactant protein B-deficient fetal mice
32. Overexpression of surfactant protein-C mature peptide causes neonatal lethality in transgenic mice
33. A mutation in the surfactant protein C gene associated with familial interstitial lung disease
34. Biosynthesis of surfactant protein C: Characterization of aggresome formation by EGFP chimeras containing propeptide mutants lacking conserved cysteine residues
35. Aggresomes: A cellular response to misfolded proteins
36. Structure and functions of a dimeric form of surfactant protein SP-C: A Fourier transform infrared and surfactometry study
37. Altered stability of pulmonary surfactant in SP-C-deficient mice
38. Very low surfactant protein C contents in newborn Belgian White and Blue calves with respiratory distress syndrome
39. Reduced surfactant protein C expression in full-term infants with respiratory distress syndrome
40. Membrane properties and amyloid fibril formation of lung surfactant protein C
41. Amyloid fibril formation by pulmonary surfactant protein C
42. The palmitoyl groups of lung surfactant protein C reduce unfolding into a fibrillogenic intermediate
43. Expression of thyroid transcription factor-1 (TTF-1) in fetal and neonatal human lung
44. Recombinant adenoviral vector disrupts surfactant homeostasis in mouse lung
45. Epidemiology of neonatal respiratory failure in the United States: Projections from California and New York