The Storage Lipids in Tangier Disease

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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 crystalline state were identified by polarizing microscopy in macrophages in both the spleen and liver, but not in the splenic artery. The droplets within individual cells melted sharply over...

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Pathology of Tangier disease.

Two cases of Tangier disease are described in children from families unrelated to each other. Necropsy in one case, the first to be reported in this condition, showed large collections of cholesterol-laden macrophages in tonsils, thymus, lymph nodes, and colon, and moderate numbers in pyelonephritic scars and ureter. As the storage cells may be scanty in marrow, jejunum, and liver, the rectum i...

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The iPSC Awakens ANGPTL3 in Tangier Disease

Patients with genetic mutation of ATP-binding Cassette Transporter A1 (ABCA1), commonly known as Tangier Disease (TD), can be characterized by elevated levels of plasma triglycerides (hypertriglyceridemia) and low plasma high-density lipoprotein (HDL) levels. With current HDL therapies continuously failing in clinical trials, there is no cure for TD other than maintaining a very low fat diet. T...

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Postprandial hypertriglyceridaemia in patients with Tangier disease.

BACKGROUND Tangier disease (TD) is the phenotypic expression of rare familial syndromes with mutations in the ABCA1 transporter. TD results in extremely low high density lipoprotein (HDL) cholesterol and reduced low density lipoprotein cholesterol, with normal or mildly increased fasting triglyceride (TG) concentrations. Although there is a close relation between HDL cholesterol values and athe...

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Tangier disease: a structural defect in apolipoprotein A-I (apoA-I Tangier).

Tangier disease is a familial disorder characterized by orange tonsils, cholesterol ester deposition in reticuloendothelial cells, abnormal chylomicron remnants, and a marked reduction in high density lipoproteins. Plasma concentrations of the apolipoproteins apo-A-I and apoA-II in patients with Tangier disease are approximately 1% and 7% of those in normal subjects, respectively. Previous stud...

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ژورنال

عنوان ژورنال: Journal of Clinical Investigation

سال: 1977

ISSN: 0021-9738

DOI: 10.1172/jci108727