Biomaterial Database

On the pathogenesis of acquired hypo-beta-lipoproteinemia. A case associated with sideroblastic anemia. 1982

K S Bjerve, and S A Evensen, and S Stray-Pedersen, and S Skrede

A 71-year-old previously hypercholesterolemic woman developed gradually severe hypocholesterolemia of 1.55 mmol/l. Simultaneously she developed progressive sideroblastic anemia, altered erythrocyte morphology and defective platelet function. Hypo-beta-lipoproteinemia was demonstrated with abnormal lipid composition of both very low density (VLDL), low density (LDL) and high density lipoprotein (HDL) fractions. Other plasma lipids, as well as erythrocyte lipids, were normal. The fractional catabolic rate of homologous 125I-LDL was increased to 4-5-fold, while its rate of synthesis was normal. The patient's serum contained autoantibodies directed against LDL. It is concluded that her hypo-beta-lipoproteinemia was due to autoantibodies towards LDL causing an increased catabolism without any concomitant effect on the rate of LDL synthesis. It is suggested that acquired hypo-beta-lipoproteinemia can be subdivided into two types, one in which the primary defect is an increased catabolism due to immunoelimination, and another in which the primary defect is a decreased rate of LDL synthesis.

UI	MeSH Term	Description	Entries
D006995	Hypobetalipoproteinemias	Conditions with abnormally low levels of BETA-LIPOPROTEINS (low density lipoproteins or LDL) in the blood. It is defined as LDL values equal to or less than the 5th percentile for the population. They include the autosomal dominant form involving mutation of the APOLIPOPROTEINS B gene, and the autosomal recessive form involving mutation of the microsomal triglyceride transfer protein. All are characterized by low LDL and dietary fat malabsorption.	Familial Hypobetalipoproteinemia,Hypo beta Lipoproteinemia,Hypobetalipoproteinemia,Hypo beta Lipoproteinemias
D007009	Hypolipoproteinemias	Conditions with abnormally low levels of LIPOPROTEINS in the blood. This may involve any of the lipoprotein subclasses, including ALPHA-LIPOPROTEINS (high-density lipoproteins); BETA-LIPOPROTEINS (low-density lipoproteins); and PREBETA-LIPOPROTEINS (very-low-density lipoproteins).	Hypolipoproteinemia,Hypoprebetalipoproteinemia
D008075	Lipoproteins, HDL	A class of lipoproteins of small size (4-13 nm) and dense (greater than 1.063 g/ml) particles. HDL lipoproteins, synthesized in the liver without a lipid core, accumulate cholesterol esters from peripheral tissues and transport them to the liver for re-utilization or elimination from the body (the reverse cholesterol transport). Their major protein component is APOLIPOPROTEIN A-I. HDL also shuttle APOLIPOPROTEINS C and APOLIPOPROTEINS E to and from triglyceride-rich lipoproteins during their catabolism. HDL plasma level has been inversely correlated with the risk of cardiovascular diseases.	High Density Lipoprotein,High-Density Lipoprotein,High-Density Lipoproteins,alpha-Lipoprotein,alpha-Lipoproteins,Heavy Lipoproteins,alpha-1 Lipoprotein,Density Lipoprotein, High,HDL Lipoproteins,High Density Lipoproteins,Lipoprotein, High Density,Lipoprotein, High-Density,Lipoproteins, Heavy,Lipoproteins, High-Density,alpha Lipoprotein,alpha Lipoproteins
D008077	Lipoproteins, LDL	A class of lipoproteins of small size (18-25 nm) and light (1.019-1.063 g/ml) particles with a core composed mainly of CHOLESTEROL ESTERS and smaller amounts of TRIGLYCERIDES. The surface monolayer consists mostly of PHOSPHOLIPIDS, a single copy of APOLIPOPROTEIN B-100, and free cholesterol molecules. The main LDL function is to transport cholesterol and cholesterol esters to extrahepatic tissues.	Low-Density Lipoprotein,Low-Density Lipoproteins,beta-Lipoprotein,beta-Lipoproteins,LDL(1),LDL(2),LDL-1,LDL-2,LDL1,LDL2,Low-Density Lipoprotein 1,Low-Density Lipoprotein 2,LDL Lipoproteins,Lipoprotein, Low-Density,Lipoproteins, Low-Density,Low Density Lipoprotein,Low Density Lipoprotein 1,Low Density Lipoprotein 2,Low Density Lipoproteins,beta Lipoprotein,beta Lipoproteins
D008079	Lipoproteins, VLDL	A class of lipoproteins of very light (0.93-1.006 g/ml) large size (30-80 nm) particles with a core composed mainly of TRIGLYCERIDES and a surface monolayer of PHOSPHOLIPIDS and CHOLESTEROL into which are imbedded the apolipoproteins B, E, and C. VLDL facilitates the transport of endogenously made triglycerides to extrahepatic tissues. As triglycerides and Apo C are removed, VLDL is converted to INTERMEDIATE-DENSITY LIPOPROTEINS, then to LOW-DENSITY LIPOPROTEINS from which cholesterol is delivered to the extrahepatic tissues.	Pre-beta-Lipoprotein,Prebeta-Lipoprotein,Prebeta-Lipoproteins,Very Low Density Lipoprotein,Very-Low-Density Lipoprotein,Very-Low-Density Lipoproteins,Lipoprotein VLDL II,Lipoproteins, VLDL I,Lipoproteins, VLDL III,Lipoproteins, VLDL1,Lipoproteins, VLDL2,Lipoproteins, VLDL3,Pre-beta-Lipoproteins,Lipoprotein, Very-Low-Density,Lipoproteins, Very-Low-Density,Pre beta Lipoprotein,Pre beta Lipoproteins,Prebeta Lipoprotein,Prebeta Lipoproteins,VLDL Lipoproteins,VLDL1 Lipoproteins,VLDL2 Lipoproteins,VLDL3 Lipoproteins,Very Low Density Lipoproteins
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006937	Hypercholesterolemia	A condition with abnormally high levels of CHOLESTEROL in the blood. It is defined as a cholesterol value exceeding the 95th percentile for the population.	Hypercholesteremia,Elevated Cholesterol,High Cholesterol Levels,Cholesterol Level, High,Cholesterol Levels, High,Cholesterol, Elevated,Cholesterols, Elevated,Elevated Cholesterols,High Cholesterol Level,Hypercholesteremias,Hypercholesterolemias,Level, High Cholesterol,Levels, High Cholesterol
D000368	Aged	A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available.	Elderly
D000756	Anemia, Sideroblastic	Anemia characterized by the presence of erythroblasts containing excessive deposits of iron in the marrow.	Anemias, Sideroblastic,Sideroblastic Anemia,Sideroblastic Anemias