Abnormal intracellular lipid processing contributes to fat malabsorption in cystic fibrosis patients

American Journal of Physiology - Gastrointestinal and Liver Physiology

Volumn 290, Issue 4, 2006, Pages

  Peretti, Noël ^a   Roy, Claude C ^a   Drouin, Eric ^a   Seidman, Ernest ^a   Brochu, Pierre ^a   Casimir, Georges ^b   Levy, Emile ^a  

^a UNIVERSITY OF MONTREAL   (Canada)

^b HOP UNIV DES ENFANTS REINE FABIOLA   (Belgium)

Author keywords

Apolipoprotein B 48; Cystic fibrosis transmembrane conductance regulator; Fatty acids; Lipoproteins; Microsomal triglyceride transfer protein

Indexed keywords

APOLIPOPROTEIN B48; CHOLESTEROL ESTER; MICROSOMAL TRIGLYCERIDE TRANSFER PROTEIN; PALMITIC ACID; PHOSPHOLIPID; TRIACYLGLYCEROL;

ADOLESCENT; ARTICLE; CLINICAL ARTICLE; CONTROLLED STUDY; CULTURE MEDIUM; CYSTIC FIBROSIS; DIGESTIVE SYSTEM FUNCTION DISORDER; DUODENUM; ESTERIFICATION; HUMAN; HUMAN TISSUE; INCUBATION TIME; INTESTINE BIOPSY; ISOTOPE LABELING; LIPID ABSORPTION; LIPID TRANSPORT; MALABSORPTION; PRIORITY JOURNAL; PROTEIN ASSEMBLY; PROTEIN SYNTHESIS; STATISTICAL SIGNIFICANCE;

ADOLESCENT; CYSTIC FIBROSIS; FATS; FEMALE; HUMANS; INTESTINAL ABSORPTION; INTESTINES; INTRACELLULAR FLUID; LIPID METABOLISM; MALABSORPTION SYNDROMES; MALE;

EID: 33645836740     PISSN: 01931857     EISSN: 15221547     Source Type: Journal    
DOI: 10.1152/ajpgi.00332.2005     Document Type: Article

References (39)

1. Bertrand CA and Frizzell RA. The role of regulated CFTR trafficking in epithelial secretion. Am J Physiol Cell Physiol 285: C1-C18, 2003.
2. Carroccio A, Pardo F, Montalto G, Iapichino L, Soresi M, Averna MR, Iacono G, and Notarbartolo A. Use of famotidine in severe exocrine pancreatic insufficiency with persistent maldigestion on enzymatic replacement therapy. A long-term study in cystic fibrosis. Dig Dis Sci 37: 1441-1446, 1992.
3. Eggermont E and De Boeck K. Small-intestinal abnormalities in cystic fibrosis patients. Eur J Pediatr 150: 824-828, 1991.
4. Fisher EA, Zhou M, Mitchell DM, Wu X, Omura S, Wang H, Goldberg AL, and Ginsberg HN. The degradation of apolipoprotein B100 is mediated by the ubiquitin-proteasome pathway and involves heat shock protein 70. J Biol Chem 272: 20427-20434, 1997.
5. Frase LL, Strickland AD, Kachel GW, and Krejs GJ. Enhanced glucose absorption in the jejunum of patients with cystic fibrosis. Gastroenterology 88: 478-484, 1985.
6. Grubb BR and Gabriel SE. Intestinal physiology and pathology in gene-targeted mouse models of cystic fibrosis. Am J Physiol Gastrointest Liver Physiol 273: G258-G266, 1997.
7. Hardcastle J, Harwood MD, and Taylor CJ. Small intestinal glucose absorption in cystic fibrosis: a study in human and transgenic ΔF508 cystic fibrosis mouse tissues. J Pharm Pharmacol 56: 329-338, 2004.
8. Jones B, Jones EL, Bonney SA, Patel HN, Mensenkamp AR, Eichenbaum-Voline S, Rudling M, Myrdal U, Annesi G, Naik S, Meadows N, Quattrone A, Islam SA, Naoumova RP, Angelin B, Infante R, Levy E, Roy CC, Freemont PS, Scott J, and Shoulders CC. Mutations in a Sar1 GTPase of COPII vesicles are associated with lipid absorption disorders. Nat Genet 34: 29-31, 2003.
9. Kopelman H, Durie P, Gaskin K, Weizman Z, and Forstner G. Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis. N Engl J Med 312: 329-334, 1985.
10. Levy E, Beaulieu JF, Delvin E, Seidman E, Yotov W, Basque JR, and Menard D. Human crypt intestinal epithelial cells are capable of lipid production, apolipoprotein synthesis, and lipoprotein assembly. J Lipid Res 41: 12-22, 2000.
11. Levy E, Garofalo C, Thibault L, Dionne S, Daoust L, Lepage G, and Roy CC. Intraluminal and intracellular phases of fat absorption are impaired in essential fatty acid deficiency. Am J Physiol Gastrointest Liver Physiol 262: G319-G326, 1992.
12. Levy E, Lepage G, Bendayan M, Ronco N, Thibault L, Galeano N, Smith L, and Roy CC. Relationship of decreased hepatic lipase activity and lipoprotein abnormalities to essential fatty acid deficiency in cystic fibrosis patients. J Lipid Res 30: 1197-1209, 1989.
13. Levy E, Marcel Y, Deckelbaum RJ, Milne R, Lepage G, Seidman E, Bendayan M, and Roy CC. Intestinal apoB synthesis, lipids, and lipoproteins in chylomicron retention disease. J Lipid Res 28: 1263-1274, 1987.
14. Levy E, Mehran M, and Seidman E. Caco-2 cells as a model for intestinal lipoprotein synthesis and secretion. FASEB J 9: 626-635, 1995.
15. Levy E, Menard D, Delvin E, Stan S, Mitchell G, Lambert M, Ziv E, Feoli-Fonseca JC, and Seidman E. The polymorphism at codon 54 of the FABP2 gene increases fat absorption in human intestinal explants. J Biol Chem 276: 39679-39684, 2001.
16. Levy E, Roy C, Lacaille F, Lambert M, Messier M, Gavino V, Lepage G, and Thibault L. Lipoprotein abnormalities associated with cholesteryl ester transfer activity in cystic fibrosis patients: the role of essential fatty acid deficiency. Am J Clin Nutr 57: 573-579, 1993.
17. Levy E, Roy CC, Thibault L, Bonin A, Brochu P, and Seidman EG. Variable expression of familial heterozygous hypobetalipoproteinemia: transient malabsorption during infancy. J Lipid Res 35: 2170-2177, 1994.
18. Levy E, Stan S, Delvin E, Menard D, Shoulders C, Garofalo C, Slight I, Seidman E, Mayer G, and Bendayan M. Localization of microsomal triglyceride transfer protein in the Golgi: possible role in the assembly of chylomicrons. J Biol Chem 277: 16470-16477, 2002.
19. Levy E, Stan S, Garofalo C, Delvin EE, Seidman EG, and Menard D. Immunolocalization, ontogeny, and regulation of microsomal triglyceride transfer protein in human fetal intestine. Am J Physiol Gastrointest Liver Physiol 280: G563-G571, 2001.
20. Lowry OH, Rosebrough NJ, Farr AL, and Randall RJ. Protein measurement with the Folin phenol reagent. J Biol Chem 193: 265-275, 1951.
21. +/glucose cotransporter and CFTR in Caco-2 cells: relevance to cystic fibrosis. J Cell Physiol 176: 472-481, 1998.
22. Marcil V, Delvin E, Seidman E, Poitras L, Zoltowska M, Garofalo C, and Levy E. Modulation of lipid synthesis, apolipoprotein biogenesis, and lipoprotein assembly by butyrate. Am J Physiol Gastrointest Liver Physiol 283: G340-G346, 2002.
23. Ménard D, Arsenault P, and Pothier P. Biologic effects of epidermal growth factor in human fetal jejunum. Gastroenterology 94: 656-663, 1988.
24. Pencharz PB and Durie PR. Nutritional management of cystic fibrosis. Annu Rev Nutr 13: 111-136, 1993.
25. Raabe M, Flynn LM, Zlot CH, Wong JS, Veniant MM, Hamilton RL, and Young SG. Knockout of the abetalipoproteinemia gene in mice: reduced lipoprotein secretion in heterozygotes and embryonic lethality in homozygotes. Proc Natl Acad Sci USA 95: 8686-8691, 1998.
26. Raabe M, Veniant MM, Sullivan MA, Zlot CH, Bjorkegren J, Nielsen LB, Wong JS, Hamilton RL, and Young SG. Analysis of the role of microsomal triglyceride transfer protein in the liver of tissue-specific knockout mice. J Clin Invest 103: 1287-1298, 1999.
27. Riordan JR, Rommens JM, Kerem B, Alon N, Rozmahel R, Grzelczak Z, Zielenski J, Lok S, Plavsic N, Chou and JL. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 245: 1066-1073, 1989.
28. Rose MC. Epithelial mucous glycoproteins and cystic fibrosis. Horm Metab Res 20: 601-608, 1988.
29. Roy CC, Weber AM, Morin CL, Combes JC, Nussle D, Megevand A, and Lasalle R. Abnormal biliary lipid composition in cystic fibrosis. Effect of pancreatic enzymes. N Engl J Med 297: 1301-1305, 1977.
30. Sharp D, Blinderman L, Combs KA, Kienzle B, Ricci B, Wager-Smith K, Gil CM, Turck CW, Bouma ME, and Rader DJ. Cloning and gene defects in microsomal triglyceride transfer protein associated with abetalipoproteinaemia. Nature 365: 65-69, 1993.
31. Siddiqi SA, Gorelick FS, Mahan JT, and Mansbach CM. COPII proteins are required for Golgi fusion but not for endoplasmic reticulum budding of the prechylomicron transport vesicle. J Cell Sci 116: 415-427, 2003.
32. Slight I, Bendayan M, Malo C, Delvin E, Lambert M, and Levy E. Identification of microsomal triglyceride transfer protein in intestinal brush-border membrane. Exp Cell Res 300: 11-22, 2004.
33. Stelzner M, Somasundaram S, Lee SP, and Kuver R. Ileal mucosal bile acid absorption is increased in Cftr knockout mice. BMC Gastroenterol 1: 10, 2001.
34. Strandvik B, Gronowitz E, Enlund F, Martinsson T, and Wahlstrom J. Essential fatty acid deficiency in relation to genotype in patients with cystic fibrosis. J Pediatr 139: 650-655, 2001.
35. Tso P. Intestinal lipid absorption. In: Physiology of the Gastrointestinal Tract, edited by John LR. New York: Raven, 1994, p. 1867-1907.
36. Wetterau JR and Zilversmit DB. A triglyceride and cholesteryl ester transfer protein associated with liver microsomes. J Biol Chem 259: 10863-10866, 1984.
37. Wilchansky M, Pencharz PB, and Durie PR. Nutritional management of cystic fibrosis in pediatric nutrition. In: Pediatric Nutrition, edited by Reifen R, Lerner A, Branski D, and Heymans H. Basel: Karger, 1998, p. 182-209.
38. Yeung SJ, Chen SH, and Chan L. Ubiquitin-proteasome pathway mediates intracellular degradation of apolipoprotein B. Biochemistry 35: 13843-13848, 1996.
39. Zemel BS, Kawchak DA, Cnaan A, Zhao H, Scanlin TF, and Stallings VA. Prospective evaluation of resting energy expenditure, nutritional status, pulmonary function, and genotype in children with cystic fibrosis. Pediatr Res 40: 578-586, 1996.