Inherited Disorders of Complex Lipid Metabolism: A Clinical Review

Lipids are important cellular components providing many essential functions. To fulfill these various functions evolution has selected for a diverse set of lipids and this diversity is seen at the organismal, cellular and subcellular level. Understanding how cells maintain this complex lipid organization is a very challenging problem, which for lipids, is not easily addressed using biochemical and genetic techniques. Therefore, chemical tools have an important role to play in our quest to understand the complexities of lipid metabolism. Here we discuss new chemical tools to study lipids, their distribution and metabolism with increased spatial and temporal resolution.

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Alterations of complex lipid metabolism in tumorigenic virus transformed cell lines

Advances in Enzyme Regulation ◽

10.1016/0065-2571(73)90018-6 ◽

1973 ◽

Vol 11 ◽

pp. 231-251 ◽

Cited By ~ 2

Author(s):

Roscoe O. Brady ◽

Peter H. Fishman ◽

Peter T. Mora

Keyword(s):

Lipid Metabolism ◽

Cell Lines ◽

Transformed Cell ◽

Complex Lipid ◽

Transformed Cell Lines

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Radioisotope-labelled substrates in enzyme assays with special reference to the preparation of radiolabelled glycosphingolipids and their use in the diagnosis of inborn errors of complex lipid metabolism

Pathology ◽

10.1016/s0031-3025(16)38713-x ◽

1977 ◽

Vol 9 (1) ◽

pp. 84-85

Author(s):

W.F. Carey ◽

A. Poulos ◽

A.C. Pollard

Keyword(s):

Lipid Metabolism ◽

Special Reference ◽

Enzyme Assays ◽

Inborn Errors ◽

Complex Lipid

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A Cytochemical Study of Glucose-6-Phosphatase (G-6-PASE) in Cells Participating in Atherogenesis of Rabbit Aorta

Proceedings, annual meeting, Electron Microscopy Society of America ◽

10.1017/s0424820100115924 ◽

1975 ◽

Vol 33 ◽

pp. 460-461

Author(s):

Sidney D. Kobernick ◽

Edna A. Elfont ◽

Neddra L. Brooks

Keyword(s):

Lipid Metabolism ◽

New Zealand ◽

Aortic Wall ◽

Rabbit Aorta ◽

Plaque Formation ◽

Metabolic Changes ◽

Atherosclerotic Plaques ◽

Cytochemical Study ◽

Cellular Alteration ◽

New Zealand White Rabbits

This cytochemical study was designed to investigate early metabolic changes in the aortic wall that might lead to or accompany development of atherosclerotic plaques in rabbits. The hypothesis that the primary cellular alteration leading to plaque formation might be due to changes in either carbohydrate or lipid metabolism led to histochemical studies that showed elevation of G-6-Pase in atherosclerotic plaques of rabbit aorta. This observation initiated the present investigation to determine how early in plaque formation and in which cells this change could be observed.Male New Zealand white rabbits of approximately 2000 kg consumed normal diets or diets containing 0.25 or 1.0 gm of cholesterol per day for 10, 50 and 90 days. Aortas were injected jin situ with glutaraldehyde fixative and dissected out. The plaques were identified, isolated, minced and fixed for not more than 10 minutes. Incubation and postfixation proceeded as described by Leskes and co-workers.

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