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May 1959, Vol 97, No. 5_PART_II >
Article | May 1959

Plasma Lipoproteins in the Lipoidoses:  Changes After a Special Dietary Regimen FREE

MARION BARCLAY, Ph.D.; D. NACKIE CALATHES, B.S.; HAROLD W. DARGEON, M.D.
[+] Author Affiliations

This investigation was supported in part by the American Cancer Society, the Atomic Energy Commission (Contract Number AT (30-1) 910), the National Cancer Institute, Public Health Service (Research Grants C-471 and CY-3192), and the United States Armed Forces Special Weapons Project (DA-49-007-MD-669).


AMA Am J Dis Child. 1959;97(5_PART_II):719-729. doi:10.1001/archpedi.1959.02070010721011.
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Introduction  A number of investigators, over the years, have demonstrated that in amaurotic family idiocy (Tay-Sachs' disease) some abnormality in lipid metabolism is likely.1 If this disease may be classified as another resulting from an "inborn error of metabolism," the indications at present are that the error is intimately related to the lipids; whether through genetic channels (suggested by the name and incidence in families), through embryologic anomalies, or through post-natal factors, is not definitely known.Recent advances in our knowledge of lipid metabolism promise to further the solution to this problem just as, for example, the newer developments of amino acid metabolism have elucidated the abnormality in phenylketonuria.2 It is now quite generally accepted that lipids, whether dietary or metabolic, are transported, at least in man, as lipid-protein or lipid-carbohydrate complexes or both. The various lipids are associated with albumin and the globulins, characterized electrophoretically as alpha-globulin

REFERENCES

1 +
Van Creveld, S.:  The Lipoidoses , in Advances in Pediatrics, vol. VI , edited by S. Z. Levine and others. Chicago, 1953;. The Year Book Publishers, Inc., pp. 190-242.
2 +
Knox, W. E., and Hsia, D. Y.-Y.:  Pathogenetic Problems in Phenylketonuria . Am. J. Med. 22:687-702, 1957;.
3 +
Oncley, J. L., and Gurd, F. R. N.:  The Lipoproteins of Human Plasma , in Blood Cells and Plasma Proteins , edited by J. L. Tullis, Academic Press, Inc., 337-357, 1953;.
4 +
Korn, E.:  Clearing Factor, A Heparin Activated Lipoprotein Lipase. II. Substrate Specificity and Activation of Coconut Oil . J Biol. Chem. 215:15-26, 1955;.
5 +
Dargeon, H. W.; Barclay, M.; Calathes, D. N., and Brownell, M. J.:  Observations on the Plasma Lipoproteins in a Case of Tay-Sachs' Disease . The Jour. of Pediatrics 52:48-57, 1958;.
6 +
Lever, W. F.; Gurd, F. R. N.; Uroma, E.; Brow, R. K.; Barnes, B. A.; Schmid, K., and Schultz, E. L.:  Chemical, Clinical, and Immunological Studies on the Products of Human Plasma Fractionation. XL. Quantitative Separation and Determination of the Protein Components in Small Amounts of Normal Human Plasma . J. Clin. Investigation 30:99-111, 1951;.
7 +
Cohn, E. J.; Gurd, F. R. N.; Surgenor, D. M.; Barnes, B. A.; Brown, R. K.; Derouaux, G.; Gillespie, J. M.; Kahnt, F. W.; Lever, W. F.; Liu, C. H.; Mittelman, D.; Mouton, R. F.; Schmid, K., and Uroma, E.:  A System for the Separation of the Components of Human Blood: Quantitative Procedures for the Separation of the Protein Components of Human Plasma . J. Am. Chem. Soc. 72:465-474, 1950;.
8 +
Pedersen, K. O.:  Ultracentrifugal Studies on Serum and Serum Fractions . J. Phys. and Colloid Chem. 51:156, 1947;.
9 +
Lindgren, F. T.; Elliott, H. A., and Gofman, J. W.:  The Ultracentrifugal Characterization and Isolation of Human Blood Lipids and Lipoproteins, with Applications to the Study of Atherosclerosis . J. Phys. and Colloid Chem. 55:80-93, 1950;.
10 +
Barclay, M.; Cogin, G. E.; Escher, G. C.; Kaufman, R. J.; Kidder, E. D., and Petermann, M. L.:  Human Plasma Lipoproteins. I. In Normal Women and in Women with Advanced Carcinoma of the Breast . Cancer 8:253-260, 1955;.
11 +
Russ, E. M.; Eder, H. A., and Barr, D. P.:  Protein-Lipid Relationships in Human Plasma. I. In Normal Individuals . Am. J. Med. 11:468-479, 1951;.
12 +
Oncley, J. L.; Gurd, F. R. N., and Melin, M.:  Preparation and Properties of Serum and Plasma Proteins. XXV. Composition and Properties of Human Serum Beta-Lipoprotein . J. Am. Chem. Soc. 72:458-464, 1950;.
13 +
Bickel, H.; Gerrard, J., and Hickmans, E. M.:  The Influence of Phenylalanine Intake on the Chemistry and Behavior of a Phenylketonuric Child . Acta Paediat . 43:64-67, 1954;.
14 +
Horner, F. A., and Stremer, C. W.:  Effect of a Phenylalanine-Restricted Diet on Patients with Phenylketonuria . J. A. M. A. 161:1628-1630, 1956;.
15 +
Hartroft, W. S.:  Effects of Various Types of Lipids in Experimental Hypolipotropic Diets . Fed. Proc. 14:655-660, 1955;.
16 +
Cochrane, G. C.; Michaels, G. D., and Kinsell, L. W.:  Dietary Modifications of Plasma Cholesterol and Phospholipid Levels in Diabetic Patients. The Effects of Mixed Diets High in Vegetable Fat . J. Clin. Nutrition 1:295-298, 1953;.
17 +
Ahrens, E. H.; Blankenhorn, D. H., and Tsaltas, T. T.:  Effect on Human Serum Lipids of Substituting Plant for Animal Fat in Diet . Proc. Soc. Exper. Biol. & Med. 86:872-878, 1954;.
18 +
Deuel, H. J.:  Fat as a Required Nutrient of the Diet . Fed. Proc. 14:639-649, 1955;.
19 +
Gofman, J. W.; Glazier, F.; Tamplin, A.; Strisower, B., and deLalla, O.:  Lipoproteins, Coronary Heart Disease, and Atherosclerosis . Physiol. Rev. 34:589-607, 1954;.
20 +
Pansky, B., and Lee, R.:  Niemann-Pick Disease in a Boy of 16 Months . J. Pediat. 51:290-299, 1957;.
21 +
Kaufman, R. J.; Barclay, M.; Kidder, E. D.; Escher, G. C., and Petermann, M. L.:  Human Plasma Lipoproteins. II. The Effect of Osseous Metastases in Patients with Advanced Carcinoma of the Breast . Cancer 8:888-889, 1955;.
22 +
Biesele, J. J.:  Antagonistic Effects of 6-Mercaptopurine and Coenzyme A on Mitochondria and Mitosis in Tissue Culture . J. Biophys. and Biochem. Cytol. 1:119-126, 1955;.
23 +
Clarke, D. A.: Personal communication.

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References

1 +
Van Creveld, S.:  The Lipoidoses , in Advances in Pediatrics, vol. VI , edited by S. Z. Levine and others. Chicago, 1953;. The Year Book Publishers, Inc., pp. 190-242.
2 +
Knox, W. E., and Hsia, D. Y.-Y.:  Pathogenetic Problems in Phenylketonuria . Am. J. Med. 22:687-702, 1957;.
3 +
Oncley, J. L., and Gurd, F. R. N.:  The Lipoproteins of Human Plasma , in Blood Cells and Plasma Proteins , edited by J. L. Tullis, Academic Press, Inc., 337-357, 1953;.
4 +
Korn, E.:  Clearing Factor, A Heparin Activated Lipoprotein Lipase. II. Substrate Specificity and Activation of Coconut Oil . J Biol. Chem. 215:15-26, 1955;.
5 +
Dargeon, H. W.; Barclay, M.; Calathes, D. N., and Brownell, M. J.:  Observations on the Plasma Lipoproteins in a Case of Tay-Sachs' Disease . The Jour. of Pediatrics 52:48-57, 1958;.
6 +
Lever, W. F.; Gurd, F. R. N.; Uroma, E.; Brow, R. K.; Barnes, B. A.; Schmid, K., and Schultz, E. L.:  Chemical, Clinical, and Immunological Studies on the Products of Human Plasma Fractionation. XL. Quantitative Separation and Determination of the Protein Components in Small Amounts of Normal Human Plasma . J. Clin. Investigation 30:99-111, 1951;.
7 +
Cohn, E. J.; Gurd, F. R. N.; Surgenor, D. M.; Barnes, B. A.; Brown, R. K.; Derouaux, G.; Gillespie, J. M.; Kahnt, F. W.; Lever, W. F.; Liu, C. H.; Mittelman, D.; Mouton, R. F.; Schmid, K., and Uroma, E.:  A System for the Separation of the Components of Human Blood: Quantitative Procedures for the Separation of the Protein Components of Human Plasma . J. Am. Chem. Soc. 72:465-474, 1950;.
8 +
Pedersen, K. O.:  Ultracentrifugal Studies on Serum and Serum Fractions . J. Phys. and Colloid Chem. 51:156, 1947;.
9 +
Lindgren, F. T.; Elliott, H. A., and Gofman, J. W.:  The Ultracentrifugal Characterization and Isolation of Human Blood Lipids and Lipoproteins, with Applications to the Study of Atherosclerosis . J. Phys. and Colloid Chem. 55:80-93, 1950;.
10 +
Barclay, M.; Cogin, G. E.; Escher, G. C.; Kaufman, R. J.; Kidder, E. D., and Petermann, M. L.:  Human Plasma Lipoproteins. I. In Normal Women and in Women with Advanced Carcinoma of the Breast . Cancer 8:253-260, 1955;.
11 +
Russ, E. M.; Eder, H. A., and Barr, D. P.:  Protein-Lipid Relationships in Human Plasma. I. In Normal Individuals . Am. J. Med. 11:468-479, 1951;.
12 +
Oncley, J. L.; Gurd, F. R. N., and Melin, M.:  Preparation and Properties of Serum and Plasma Proteins. XXV. Composition and Properties of Human Serum Beta-Lipoprotein . J. Am. Chem. Soc. 72:458-464, 1950;.
13 +
Bickel, H.; Gerrard, J., and Hickmans, E. M.:  The Influence of Phenylalanine Intake on the Chemistry and Behavior of a Phenylketonuric Child . Acta Paediat . 43:64-67, 1954;.
14 +
Horner, F. A., and Stremer, C. W.:  Effect of a Phenylalanine-Restricted Diet on Patients with Phenylketonuria . J. A. M. A. 161:1628-1630, 1956;.
15 +
Hartroft, W. S.:  Effects of Various Types of Lipids in Experimental Hypolipotropic Diets . Fed. Proc. 14:655-660, 1955;.
16 +
Cochrane, G. C.; Michaels, G. D., and Kinsell, L. W.:  Dietary Modifications of Plasma Cholesterol and Phospholipid Levels in Diabetic Patients. The Effects of Mixed Diets High in Vegetable Fat . J. Clin. Nutrition 1:295-298, 1953;.
17 +
Ahrens, E. H.; Blankenhorn, D. H., and Tsaltas, T. T.:  Effect on Human Serum Lipids of Substituting Plant for Animal Fat in Diet . Proc. Soc. Exper. Biol. & Med. 86:872-878, 1954;.
18 +
Deuel, H. J.:  Fat as a Required Nutrient of the Diet . Fed. Proc. 14:639-649, 1955;.
19 +
Gofman, J. W.; Glazier, F.; Tamplin, A.; Strisower, B., and deLalla, O.:  Lipoproteins, Coronary Heart Disease, and Atherosclerosis . Physiol. Rev. 34:589-607, 1954;.
20 +
Pansky, B., and Lee, R.:  Niemann-Pick Disease in a Boy of 16 Months . J. Pediat. 51:290-299, 1957;.
21 +
Kaufman, R. J.; Barclay, M.; Kidder, E. D.; Escher, G. C., and Petermann, M. L.:  Human Plasma Lipoproteins. II. The Effect of Osseous Metastases in Patients with Advanced Carcinoma of the Breast . Cancer 8:888-889, 1955;.
22 +
Biesele, J. J.:  Antagonistic Effects of 6-Mercaptopurine and Coenzyme A on Mitochondria and Mitosis in Tissue Culture . J. Biophys. and Biochem. Cytol. 1:119-126, 1955;.
23 +
Clarke, D. A.: Personal communication.

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