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Tangier Disease Summary

Description: An autosomal recessively inherited disorder caused by mutation of ATP-BINDING CASSETTE TRANSPORTERS involved in cellular cholesterol removal (reverse-cholesterol transport). It is characterized by near absence of ALPHA-LIPOPROTEINS (high-density lipoproteins) in blood. The massive tissue deposition of cholesterol esters results in HEPATOMEGALY; SPLENOMEGALY; RETINITIS PIGMENTOSA; large orange tonsils; and often sensory POLYNEUROPATHY. The disorder was first found among inhabitants of Tangier Island in the Chesapeake Bay, MD.

Also Known As: HDLDT1; High Density Lipoprotein Deficiency, Type 1; High-Density Lipoprotein Deficiency, Tangier Type; High-Density Lipoprotein Deficiency, Type I; Neuropathy of Tangier Disease Show All >>

Networked: 362 relevant articles (10 outcomes, 23 trials/studies) for this Disease

Key Drugs and Agents for Tangier Disease

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Apolipoprotein A-I (Apolipoprotein A1) : 3 outcomes 5 studies in 88 results : IBA
  2. Cholesterol : 2 outcomes 7 studies in 124 results : IBA
  3. Apolipoproteins : 2 outcomes 2 studies in 41 results : IBA
  4. Lipoproteins (Lipoprotein) : 1 outcome 6 studies in 86 results : IBA
  5. Adenosine Triphosphate (ATP) : 1 outcome 3 studies in 71 results : IBA
  6. Apoproteins : 1 outcome 2 studies in 6 results : IBA
  7. Phosphatidylcholines (Phosphatidylcholine) : 1 outcome in 3 results : IBA
  8. Telomerase (Telomerase Reverse Transcriptase) : 1 outcome in 2 results : IBA
  9. Ca(2+) Mg(2+)-ATPase : 1 outcome in 1 result : IBA
  10. Retinaldehyde (Retinal) : 1 outcome in 1 result : IBA
Show All >>

Diseases Related to Tangier Disease

  1. Lecithin Acyltransferase Deficiency (Lecithin:Cholesterol Acyltransferase Deficiency)
  2. Atherosclerosis
  3. Macular Degeneration (Age-Related Maculopathy)
  4. Mononeuropathies (Mononeuritis Multiplex)
  5. Lamellar Ichthyosis (Harlequin Ichthyosis)
Show All >>

Key Therapies for Tangier Disease

Efficacy Chart >>
  1. Ligation : 1 study in 1 result
  2. Plasma Exchange : 2 results
  3. Coronary Artery Bypass (Coronary Artery Bypass Surgery) : 2 results
  4. Splenectomy : 1 result
  5. Anesthesia : 1 result

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