Lysosomal acid lipase over-expression disrupts lamellar body genesis and alveolar structure in the lung

International Journal of Experimental Pathology

Volumn 88, Issue 6, 2007, Pages 427-436

  Li, Yuan ^a,c   Qin, Yulin ^b   Li, Huimin ^b   Wu, Renliang ^c   Yan, Cong ^a,c   Du, Hong ^b  

^a INDIANA UNIVERSITY SCHOOL OF MEDICINE   (United States)

^b CINCINNATI CHILDREN'S HOSPITAL MEDICAL CENTER   (United States)

^c HUAZHONG UNIVERSITY OF SCIENCE AND TECHNOLOGY   (China)

Author keywords

Alveolar structure; Emphysema; Lysosomal acid lipase; Neutral lipids; Pulmonary surfactant; Transgenic mice

Indexed keywords

ACID LIPASE; CHOLESTEROL; CHOLESTEROL ESTER; DOXYCYCLINE; FATTY ACID DERIVATIVE; LUNG SURFACTANT; SURFACTANT PROTEIN A; SURFACTANT PROTEIN B; SURFACTANT PROTEIN C; TRIACYLGLYCEROL;

ANIMAL CELL; ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; GENE EXPRESSION; HYDROLYSIS; LAMELLAR BODY; LIPID DEGRADATION; LUNG ALVEOLUS; LUNG ALVEOLUS EPITHELIUM; LUNG EMPHYSEMA; LUNG LAVAGE; LUNG STRUCTURE; LYSOSOME; MACROPHAGE; MOUSE; NONHUMAN; PRIORITY JOURNAL; PROTEIN EXPRESSION; TRANSGENIC MOUSE;

ANIMALS; BRONCHOALVEOLAR LAVAGE FLUID; DOXYCYCLINE; EPITHELIAL CELLS; GENE EXPRESSION; MACROPHAGES; MICE; MICE, TRANSGENIC; MICROSCOPY, ELECTRON; PULMONARY ALVEOLI; PULMONARY EMPHYSEMA; PULMONARY SURFACTANTS; REVERSE TRANSCRIPTASE POLYMERASE CHAIN REACTION; STEROL ESTERASE;

EID: 36448962797     PISSN: 09599673     EISSN: 13652613     Source Type: Journal    
DOI: 10.1111/j.1365-2613.2007.00547.x     Document Type: Article

References (18)

1. Burton B.K., Remy W.T., Rayman L. (1984) Cholesterol ester and triglyceride metabolism in intact fibroblasts from patients with Wolman's disease and cholesterol ester storage disease. Pediatr. Res. 18, 1242 1245.
2. Clark J.C., Wert S.E., Bachurski C.J. et al. (1995) Targeted disruption of the surfactant protein B gene disrupts surfactant homeostasis, causing respiratory failure in newborn mice. Proc. Natl Acad. Sci. U.S.A. 92, 7794 7798.
3. Du H., Heur M., Duanmu M. et al. (2001a) Lysosomal acid lipase-deficient mice: depletion of white and brown fat, severe hepatosplenomegaly, and shortened life span. J. Lipid Res. 42, 489 500.
4. Du H., Schiavi S., Levine M., Mishra J., Heur M., Grabowski G. A. (2001b) Enzyme therapy for lysosomal acid lipase deficiency in the mouse. Hum. Mol. Genet. 10, 1639 1648.
5. Glasser S.W., Detmer E.A., Ikegami M., Na C.L., Stahlman M. T., Whitsett J. A. (2003) Pneumonitis and emphysema in sp-C gene targeted mice. J. Biol. Chem. 278, 14291 14298.
6. Lian X., Yan C., Yang L., Xu Y., Du H. (2004) Lysosomal acid lipase deficiency causes respiratory inflammation and destruction in the lung. Am. J. Physiol. Lung Cell Mol. Physiol. 286, L801 807.
7. Lian X., Qin Y., Hossain S.A. et al. (2005a) Overexpression of Stat3C in pulmonary epithelium protects against hyperoxic lung injury. J. Immunol. 174, 7250 7256.
8. Lian X., Yan C., Qin Y., Knox L., Li T., Du H. (2005b) Neutral lipids and peroxisome proliferator-activated receptor-[gamma] control pulmonary gene expression and inflammation-triggered pathogenesis in lysosomal acid lipase knockout mice. Am. J. Pathol. 167, 813 821.
9. Rudel L. L., Morris M. D. (1973) Determination of cholesterol using O-phthalaldehyde. J lipid Res 14, 164 366.
10. Sando G. N., Rosenbaum L. M. (1985) Human lysosomal acid lipase/cholesteryl ester hydrolase. Purification and properties of the form secreted by fibroblasts in microcarrier culture. J. Biol. Chem. 260, 15186 15193.
11. Schmitz G., Muller G. (1991) Structure and function of lamellar bodies, lipid-protein complexes involved in storage and secretion of cellular lipids. J. Lipid Res. 32, 1539 1570.
12. Tichelaar J.W., Lu W., Whitsett J.A. (2000) Conditional expression of fibroblast growth factor-7 in the developing and mature lung. J. Biol. Chem. 275, 11858 11864.
13. Whitsett J.A. (2005) Surfactant proteins in innate host defense of the lung. Biol. Neonate 88, 175 180.
14. Whitsett J.A., Weaver T.E. (2002) Hydrophobic surfactant proteins in lung function and disease. N. Engl. J. Med. 347, 2141 2148.
15. Wright J.R. (2005) Immunoregulatory functions of surfactant proteins. Nat. Rev. Immunol. 5, 58 68.
16. Yan C., Du H. (2004) Alveolus formation: what have we learned from genetic studies? J. Appl. Physiol. 97, 1543 1548.
17. -/- mice. Am. J. Pathol. 169, 916 926.
18. Yang L., Naltner A., Yan C. (2003) Overexpression of dominant negative retinoic cid receptor alpha causes alveolar abnormality in transgenic neonatal lungs. Endocrinology 144, 3004 3011.