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TANGIER DISEASE (TD) A.Saidatul

LIPID METABOLISM II. TANGIER DISEASE (TD) A.Saidatul. What is TD, and what causes it? What does TD tell us about cholesterol and atherosclerosis?. Normally …. ABC1 is present to aid cholesterol out of the cell Cholesterol then binds with Apo-A1 lipoprotein outside the cell forming HDL

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TANGIER DISEASE (TD) A.Saidatul

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  1. LIPID METABOLISM II TANGIER DISEASE (TD)A.Saidatul What is TD, and what causes it? What does TD tell us about cholesterol and atherosclerosis?

  2. Normally… • ABC1 is present to aid cholesterol out of the cell • Cholesterol then binds with Apo-A1 lipoprotein outside the cell forming HDL • HDL picks up additional lipids from LDL and VLDL lipoproteins and transfers the cholesterol to the liver, where it is processed Cholesterol is successfully transported out of cells and processed Image taken from: http://www.ncbi.nlm.nih.gov/books/bv.fcgi?call=bv.View..ShowSection&rid=gnd.section.237

  3. In Tangier Syndrome… • Cholesterol is forced to accumulate within the cell, since there is a mutation present in ABC1 Image taken from: http://www.ncbi.nlm.nih.gov/books/bv.fcgi?call=bv.View..ShowSection&rid=gnd.section.237

  4. TD is a genetic disorder • ABC1 gene is located on chromosome 9q31 • It codes for an mRNA that is 6,783 base pairs and a protein that is 2,261 amino acids in size • The protein synthesised is a transmembrane protein with 12 transmembrane domains Image taken from: http://www.ncbi.nlm.nih.gov/disease/chr9-12.html

  5. CHARACTERISTICS OF TANGIER DISEASE: • Hypertrophic orange-colour tonsils • Very low levels of HDL in the plasma • An enlarged liver and spleen • Premature coronary artery disease

  6. How does it link to atherosclerosis • This disease leads to increased cholesterol esters in the tonsils, spleen, liver, skin and lymph nodes • The discovery of this cholesterol gene has lead to a better understanding of the inverse relationship between HDL and coronary heart disease • Since it leads to atherosclerosis, it clearly shows that cholesterol is an important factor in heart disease • It is a rare disease but it demonstrates that we should be aware of our cholesterol levels

  7. Hypercholesterolemia High Cholesterol Level

  8. Hypercholesterolemia: • The normal range for total blood cholesterol is between140 and 200 mg per decilitre (mg/dL) . • Hypercholesterolemia, or (high cholesterol), occurs when there is too much cholesterol in the body. • High cholesterol raises your risk for heart disease, heart attack, and stroke.

  9. There are two types of cholesterol : • A) HDL (high-density lipoproteins), also named ("good" cholesterol) . • B)LDL (low-density lipoproteins), also named( "bad" cholesterol). • The amount of HDL relative to LDL is considered a more important indicator of your heart disease risk . Signs and Symptoms: • There usually aren't any symptoms of high cholesterol, especially in early stages. The only way to tell if your cholesterol is high is through a blood test.

  10. Risk Factors : • Some factors increase a person's risk of having high cholesterol. While some of these cannot be changed, many can be. The most important risk factors for high cholesterol are: • Being overweight or obese . • Eating a diet high in saturated fat and trans fatty acids (found in processed and fried foods) . • Not getting enough exercise . • Family history of heart disease. • High blood pressure . • Smoking . • Diabetes.

  11. Total cholesterol levels: • Desirable: Below 200 mg/dL . • Borderline high: 200 - 239 . • High: Above 240. • LDL cholesterol levels: • Optimal for people with heart disease or who are at high risk: Below 70 mg/dL. • Optimal for people at risk of heart disease: Below 100 . • Optimal: 100 - 129 . • Borderline high: 130 - 159 . • High: 160 – 189. • HDL cholesterol levels: • Poor: Below 40 mg/dL . • Acceptable: 40 – 59. • Optimal: 60 or above. • You should have liver function tests as well if you are on cholesterol-lowering medication.

  12. Preventive Care: • Most people can lower cholesterol levels by eating a well-balanced diet, getting regular exercise, and losing any excess weight. • Losing Weight • Being overweight increases risk of high cholesterol and heart disease. Even a 5- to 10-pound weight loss can lower LDL twice as much as diet alone. Weight loss often results in lower triglyceride levels and increased HDL, too. To maintain a healthy diet, you should aim for a gradual, weekly weight loss of 1/2 to 1 pound.

  13. Prognosis and Complications: • Heart disease -- high cholesterol levels more than double the risk of heart attack. Lowering cholesterol by 1% reduces the risk of coronary artery disease by 2%.

  14. Stroke -- low levels of "good" HDL cholesterol have been associated with an increased risk of stroke. • Insulin resistance -- 88% of people with low HDL and 84% with high triglycerides also have insulin resistance (which leads to high blood sugar levels). Many people with insulin resistance go on to develop diabetes.

  15. ِAny Questions?

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