About alpha high-density lipoprotein deficiency

What is alpha high-density lipoprotein deficiency?

Tangier Disease is an inherited blood disorder involving decreased concentrations of fat compounds in the blood called high-density lipoproteins (sometimes called "good cholesterol"). Large amounts of these compounds may accumulate in certain organs of the body causing tissue discoloration. In later stages, these accumulations may cause organ enlargement and/or blood circulation problems.

What are the risk factors for alpha high-density lipoprotein deficiency?

Tangier disease is caused by a mutation in the ATP-binding cassette A1 (ABCA1) gene, which produces severe high-density lipoprotein (HDL) deficiency syndrome. Tangier illness is a genetic metabolic ailment that is highly rare. It induces reverse cholesterol transport (RCT) dysfunction, which leads to cholesterol deposition in peripheral tissue cells and a variety of clinicopathological alterations, including dyslipidemia and the early beginning of atherosclerosis, terminating in cerebral infarction.

Is there a cure/medications for alpha high-density lipoprotein deficiency?

Tangier syndrome is a disorder with hereditary ailment characterized by dramatically lower blood levels of high-density lipoproteins (HDL). HDL transfers cholesterol and phospholipids, which are fats, from the body's tissues to the liver, where they are eliminated from the blood. HDL-cholesterol (HDL-C) is sometimes referred to as "good cholesterol" because it can help remove cholesterol from the walls of arteries, particularly the coronary (heart) arteries, lowering the risk of developing heart and blood vessels (cardiovascular) disease. Tangier disease patients have a considerably increased risk of cardiovascular disease due to extremely low HDL levels. Tangier syndrome has no existing specific therapies. Tangier disease treatment is supportive and focused on the specific illness symptoms in a given individual. Drugs that are known to boost high-density lipoprotein levels in healthy persons, such as estrogens, nicotinic acid, statins, or phenytoin, do not work in Tangier disease patients. People with this illness should adopt a reduced-fat (particularly saturated fat) diet and an overall healthy lifestyle to lower their risk of heart and blood vessel damage. Smoking, high blood pressure, diabetes, obesity, and excessive levels of triglycerides and homocysteine in the blood should all be treated as soon as possible. Triglycerides can be reduced by using fibrates.

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