Abstract
We have recently reported a unique case of a new type of lipid storage disorder (Duboucher et al., 1986; Rocchiccioli et al., 1987) which we have named Alkane Storage Disease. This novel human morbid entity is characterized by the visceral accumulation of odd-numbered long chain n-alkanes. These alkanes are characteristic of vegetal cuticular waxes and differ from those present in mineral oil paraffin (Lester, 1979; Levade et al., 1987). The nature of the stored compounds, their visceral distribution and the clinical anamnesis strongly suggest a dietary origin. We also discuss the hypothetical metabolic defect which may cause the n-alkane accumulation and the pathophysiological mechanism explaining their tissular distribution.
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References
Albro, P. W., and Fishbein, L., 1970, Absorption of aliphatic hydrocarbons by rats, Biochim. Biophys. Acta, 219:437.
Bandursky, E. L., and Nagy, B., 1974, Nature of aklanes in beef heart lipids, Lipids, 10:67.
Chibnall, A. C., Piper, S.H., Pollard, A., Smith, J. A. B., and Williams, E. F., 1931, The wax constituents of the apple cuticle, Biochim. J., 25:2095.
Cruickshank, B., and Thomas, J. M., 1984, Mineral oil (follicular) lipidosis: II. Histologic studies of spleen, liver, lymph nodes and bone marrow, Hum. Pathol., 15:731.
Dongay, G,, Levade, T,, Caratero, A,, Salvayre, R., Lauque, D., and Carles, P., 1985, Paraffinose alveolaire; etude cytologique et biochimique du liquide de lavage bronchiolo-alveolaire, Rev. Ma Resp., 2:231.
Duboucher, C,, Milhau, S., and Bouissou, H., 1987, Isolated amyloidos of the atrioventricular valves: a study of one case, curiously associated with diffuse storage of plant wax paraffin, Virchows Arch. A, 410:541.
Duboucher, C., Escamilla, R., Rocchiccioli, F., Negre, A., Lageron, A. and Migueres, J., 1986, Pulmonary lipogranulomatosis due to excesive consumption of apples, Chest, 90:611.
Folch, J., Lees, M., and Sloane-Stanley, G. H., 1957, A simple method the isolation and purification of total lipids from animal tissues, J. Biol. Chem., 226:497.
Heckers, H,, Melcher, F. W., Dittmar, K., and Kalinowski, H. O., 1978, Paraffin oil pneumonia. Analysis of saturated hydrocarbons in different human tissues, J. Chromatogr,, 146:91.
Kolattukudy, P. E., and Hankin, L., 1966, Metabolism of a plant wax paraffin (n-nonacosane) in the rat, J. Nutrition, 90:167.
Lester, D. E., 1979, Normal paraffins in living matter - occurence, metabolism and pathology, Prog. Fd. Nutr. Sci., 3:1.
Levade, T., Salvayre, R., Dongay, G., Dang, Q. Q., Vieu, C., Bessac, A and Douste-Blazy, L., 1987, Chemical analysis of the bronchoalveolar washing fluid in the diagnosis of liquid paraffin pneumonia, J. Clin. Chem. Clin. Biochem., 25:45.
Morice, I. M., and Shorland, F. B., 1973, Composition of the surface c apple fruits and changes during storage, J. Sci. Food Agr., 24:1331.
Morozova, N. P., and Salkova, E. G., 1970, The composition of paraffir hydrocarbons of the plant cuticular wax, Prikl. Biokhim. Microbiol., 6:697.
Nagy, B., Modzeleski, V. E., and Scott, W. M., 1969, Saturated hydroca bons in bovine liver, Biochem. J., 114:645.
Negre, A., Salvayre, R., Maret, A., Vieu, C., Bes, J-C., Borrone, C., Durand, P., and Douste-Blazy, L., 1986, Lymphoid cell lines as model for the study of Wolman’s disease: enzymatic, metabolic a ultrastructural investigations, J. Inher. Metab. Dis., 9:193.
Rocchiccioli, F., Lageron, A., and Duboucher, C., 1987, Abnormal n-noncosane storage in humans: detection by gas chromatography/mass spectrometry of tissue extracts, Biomed. Environ. Mass Spectron in press.
Sahai, P. N., and Chibnall, A. C., 1932, Wax metabolism in the leaves Brussels sprout, Biochem. J., 26:403.
Schlunegger, U. P., 1972., Distribution pattern of n-alkanes in human liver, urine ane sweat, Biochim. Biophys. Acta, 260:339.
Spencer, H., 1977, Inhalation lipoid pneumonia including liquid paraff granuloma, in: “Pathology of the lung,” Pergamon press, Oxford, New York, Toronto, Sydney, Paris, Frankfurt.
Tulliez, J. E., and Bories, G. F., 1977, Metabolism of a n-paraffin, heptadecane, in rats, Lipids, 13:110.
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© 1988 Plenum Press, New York
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Duboucher, C., Rocchiccioli, F., Nègre, A., Lageron, A., Salvayre, R. (1988). Alkane Storage Disease (Very Long Chain N-Alkanes): An Original Type of Lipid Storage of Dietary Origin from Plant Wax Hydrocarbons. In: Salvayre, R., Douste-Blazy, L., Gatt, S. (eds) Lipid Storage Disorders. NATO ASI Series, vol 150. Springer, Boston, MA. https://doi.org/10.1007/978-1-4613-1029-7_56
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DOI: https://doi.org/10.1007/978-1-4613-1029-7_56
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