Tangier disease
In vivo metabolism of proapolipoprotein A I in Tangier disease
... Tangier disease is a rare familial disorder characterized by extremely low levels of apolipoprotein A-I (apoA-I) and high density lipoproteins ...in Tangier disease there is a marked relative ...
7
Characterization of high density lipoproteins in patients heterozygous for Tangier disease
... lipoproteins, lipid storage in histiocytes of the rectal mucosa obtained from heterozygous patients has been documented. It is concluded that patients heterozygous for Tangier disease have normal high ...
12
The Tangier disease gene product ABC1 controls the cellular apolipoprotein mediated lipid removal pathway
... A major cardioprotective activity of HDL is ascribed to its role in reverse cholesterol transport, which is the flux of cholesterol from peripheral cells such as tissue macrophages, through plasma lipoproteins to the ...
8
Two Long-Term Follow-Up Cases Preventing Atherosclerosis by Hormone Replacement Therapy-CETP Deficiency and Tangier Disease
... The fifth, estrogen enhances the synthesis of Apo-A1 as a major part of HDL-C [17], leading to higher HDL-C. Finally, 17βE2 activates CETP, consequently, transporting cholesterol ester in HDL-C to LDL-C, in order to ...
5
Tangier Disease: HIGH DENSITY LIPOPROTEIN DEFICIENCY DUE TO DEFECTIVE METABOLISM OF AN ABNORMAL APOLIPOPROTEIN A I (APOA ITANGIER)
... Tangier disease is a rare familial disorder characterized by enlarged orange tonsils, transient peripheral neuropathy, hepatosplenomegaly, and lymphadenopathy, as well as striking reductions in plasma high ...
13
Studies on the Protein Defect in Tangier Disease: ISOLATION AND CHARACTERIZATION OF AN ABNORMAL HIGH DENSITY LIPOPROTEIN
... High density lipoproteins (d 1.063-1.210 g/ml) were isolated from the plasma of normal individuals (HDL) and seven homozygous patients with Tangier disease (HDLt). In Tangier patients, the ...
16
Isolation and Characterization of an Abnormal High Density Lipoprotein in Tangier Disease
... for Tangier disease (familial high density lipoprotein ...that Tangier high density lipoproteins, as isolated by ultracentrifugation, are morphologically heterogenous and contain several proteins ...
12
Platelet activating factor levels and metabolism in tangier disease: a case study
... 2. Brooks-Wilson A, Marcil M, Clee SM, Zhang LH, Roomp K, van Dam M, Yu L, Brewer C, Collins JA, Molhuizen HO, Loubser O, Ouelette BF, Fichter K, Ashbourne-Excoffon KJ, Sensen CW, Scherer S, Mott S, Denis M, Martindale ...
10
Defective regulation of phosphatidylcholine specific phospholipases C and D in a kindred with Tangier disease Evidence for the involvement of phosphatidylcholine breakdown in HDL mediated cholesterol efflux mechanisms
... heart disease and plasma levels of HDL has been attributed to the ability of HDL to take up cellular ...(Tangier disease, ...A Tangier-like PA and DG formation pattern could be induced in ...
10
Properties of the Plasma Very Low and Low Density Lipoproteins in Tangier Disease
... in Tangier disease is well established, but the properties of very low density lipoproteins (VLDL) and low density lipoproteins (LDL) in this disorder have not been well ...of Tangier and normal VLDL ...
14
The Storage Lipids in Tangier Disease: A PHYSICAL CHEMICAL STUDY
... The physical states and phase behavior of the lipids of the spleen, liver, and splenic artery from a 38-yr-old man with Tangier disease were studied. Many intracellular lipid droplets in the smectic liquid ...
11
The lipoprotein abnormality in Tangier disease: quantitation of A apoproteins
... with Tangier disease (hereditary alpha-lipoprotein ...in Tangier plasma were determined to be less than 1% and 5-7%, ...In Tangier patients, approximately 90% of the apoprotein A-I sedimented ...
12
Defective removal of cellular cholesterol and phospholipids by apolipoprotein A I in Tangier Disease
... Tangier disease is a rare genetic disorder characterized by extremely low plasma levels of HDL and apo A-I, deposition of cholesteryl esters in tissues, and a high prevalence of cardiovascular ...
11
Characterization of apolipoprotein A I and A II containing lipoproteins in a new case of high density lipoprotein deficiency resembling Tangier disease and their effects on intracellular cholesterol efflux
... Since HDL are postulated to be involved in the transport of excess cholesterol from peripheral tissues to the liver for degradation, and the ability of an HDL particle to promote cellula[r] ...
9
Effects of miglustat treatment in a patient affected by an atypical form of Tangier disease
... Case Report: A 58 year-old female, presenting with complex clinical signs (splenomegaly, dysarthria, dysphagia, ataxia, tongue enlargement, prurigo nodularis, legs lymphedema, pancytopenia and bone marrow foam cells), ...
7
The Inheritance of High Density Lipoprotein Deficiency (Tangier Disease)
... Summary Plasma concentrations of high density lipoprotein HDL, as measured by cholesterol in the ultracentrifugal fraction of D > 1.063, have been determined in 135 subjects related to t[r] ...
10
The Evolution of the Health Status and Quality of Life of Heroin Addicts Treated with Methadone in the City of Tangier, Morocco
... of Tangier and Tetouan, and subsequently spread throughout the North and Northeast zones of the country Morocco that goes from Tangier to ...are Tangier, Rabat and Casablanca. The number of patients ...
10
Application of a Geomatics Approach for the Diachronic Study of the Meditterannean Coastline Case of Tangier Bay
... In Tangier Bay as a whole, the distribution of sediments on both the coastline and seabed is generally controlled by hydrodynamic factors, as well as by the main sources of input ...in Tangier Bay could ...
17
State of compaction realized in the public landfill of Tangier and its impact on the duration of the exploitation
... landfill Tangier to host household and industrial waste in the city until 2016 Expected opening of future landfill ...the Tangier landfill, especially composition, granulometry and ...
9
Proposal of a causal model measuring the links between quality practices and Corporate Social Responsibility (CSR): Case of the manufacturing companies located in Tangier Free Zone and in the Gueznnaya industrial area in Tangier (Morocco)
... A BSTRACT : This study aims to measure the links between quality practices and Corporate Social Responsibility of the manufacturing companies located in the Gueznnaya industrial area and Tangier free zone, based ...
13