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
BARCLAY M, CALATHES DN, DARGEON HW. Plasma Lipoproteins in the Lipoidoses: Changes After a Special Dietary Regimen. AMA Am J Dis Child. 1959;97(5_PART_II):719–729. doi:10.1001/archpedi.1959.02070010721011
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