Tangier Disease on Tangier Island Virginia

Tangier Island Guide
Tangier Island: A Chesapeake Bay Island
Tangier Disease

Now is the time to start inquiring and making reservations for the up coming Season on Tangier Island as the accommodation easily fill up. Obtain information regarding boat transportation, restaurants, and Bed & Breakfasts, museum, kayaking, and more by going to: www.TangierIsland-va.com/tangier

When one thinks of Statin Drugs today and cholesterol, one needs to think Tangier Island Virginia as this is where the research began.............

What is Tangier Disease? Tangier disease is an inherited disorder characterized by significantly reduced levels of high-density lipoprotein (HDL) in the blood. HDL transports cholesterol and certain fats called phospholipids from the body's tissues to the liver, where they are removed from the blood. HDL is often referred to as "good cholesterol" because high levels of this substance reduce the chances of developing heart and blood vessel (cardiovascular) disease. Because people with Tangier disease have very low levels of HDL, they have a moderately increased risk of cardiovascular disease.

Additional signs and symptoms of Tangier disease include a slightly elevated amount of fat in the blood (mild hypertriglyceridemia); disturbances in nerve function (neuropathy); and enlarged, orange-colored tonsils. Affected individuals often develop atherosclerosis, which is an accumulation of fatty deposits and scar-like tissue in the lining of the arteries. Other features of this condition may include an enlarged spleen (splenomegaly), an enlarged liver (hepatomegaly), clouding of the clear covering of the eye (corneal clouding), and type 2 diabetes.

The Donald S. Fredrickson Papers
http://profiles.nlm.nih.gov/FF/Views/Exhibit/narrative/scientist.html

How common is Tangier disease?

Tangier disease is a rare disorder with approximately 100 cases identified worldwide. More cases are likely undiagnosed. This condition is named after an island off the coast of Virginia where the first affected individuals were identified - Teddy and Elaine Laird (Brother & Sister)
Excerpts from: A service of the U.S. National Library of Medicine http://ghr.nlm.nih.gov/condition/tangier-disease

Teddy Laird, one of the two sibblings of which research was conducted at the NIH and later the development of statin drugs.
Photo taken: June 29, 2007 by Virginia Taylor

Date:

Teddy S. Laird
October 13th, 1955 - April 18th, 2011

Obituary:

Dateline – Kinards, SC Teddy S. Laird, age 55 of 33389 Hwy 76 died Monday, April 18, 2011 at home. He was born at Tangier Island, VA and was the son of Peggy Parks Laird of the home and the late Gladden Laird. He was a former employee of Ingles and a member of Joanna Church of God. Surviving is his wife, Amelia Nabors Laird of the home; four stepsons, Reggie Nabors and Kim of Joanna; Johnnie Bunton of Joanna; Eric Bunton and Karen of Conway; Chris Hightower and Nina of Conway and seven grandchildren. He was predeceased by a sister, Elaine Evans. A memorial service will be conducted on Wednesday, April 20, 2011 at 2:00pm at Joanna Church of God. The family will receive friends Wednesday, April 20, 2011 from 1:00 to 2:00pm at the church. Condolences may be expressed to the family at www.grayfuneralhome.com Gray Funeral Home of Clinton

Tangier Island and the Laird Children at age 12 and 13
Articles reads:
Tangier Island
Tonsils May Cure Hearts
by: John Pruitt, Virginia Pilot Staff Writer

Tangier Island Water Trails Tangier Island Transportation Joyce Marie II - Onancock, Virginia Steven Thomas, Crisfield, Maryland Chesapeake Breeze, Reedville, Va. Chesapeake Bay Eagle,Crisfield, Md Sharon Kay III, Crisfield, Md. Tangier Island Restaurants Hilda Crockett's Chesapeake House Fisherman's Corner Lorraine's on Tangier Waterfront Restaurant Tangier Island Bed and Breakfasts Mimosa Vacation Rental Hilda Crockett's Chesapeake House BB Bay View Inn Bed and Breakfast Sunset Inn Bed and Breakfast Tangier Island Parks Marina Tangier Island Buggy Tours C hesapeake Bay Crab Shanty Adventures Tangier Island Eco Tours, Sunset Tours, Crabbing, or boat tours around the Island Tangier Island History Families of Tangier Island to mention a few: Crockett, Pruitt, Thomas, Park A Pictorial View of Tangier Island. Photos provided by: Neil Kaye and Virginia Taylor Click Here Additional Tangier Island Photographs Southern Living Magazine - May 2005 Tangier Island Recipes Tangier Island Real Estate Schooner Serenity and Schooner Alliance Various Tangier Videos as shown here! NPR Radio Tangier Island - Medical A Waterman's Life Tangier Island Video By: University of Richmond Video Camping and Kayaking on Tangier Island He is known as "Dr Copter" Weekly visits by Mainland Doctor The Chesapeake Breeze departs Reedville, Va on the Western Shore of the Chesapeake Bay during Tangier Island Tourism Season as does the Steven Thomas which departs from the Eastern Shore of the Lower Chesapeake Bay at Crisfield, Md. Check Schedule. Round Trip or One Way Inquiries Make Reservations. Tangier Island Information The Joyce Marie II departs Tangier Island for Onancock Virginia on the Eastern Shore of Virginia twice daily. Transportation is seasonal. Check Schedule. Round Trip or One Way Make Reservations Tangier Island Information The Sharon Kay III departs Tangier Island for Crisfield, Md.YEAR ROUND days a week in the late afternoon around 3 or 4 pm depending on what day you depart. Return trip to Tangier from Crisfield departs at 4 or 5 pm, again, depending on the day you depart. Check Schedule. Round Trip or One Way Make Reservations Tangier Island Information Bike Rentals provided by the Waterfront Restaurant Golf Cart Rentals offered by the Sunset Inn Bed and Breakfast	Received From the Tangier History Museum Publish Date: Year 1990 After forty years shrouded in mystery, the Tangier Disease is just beginning to reveal its secrets. Perhaps, there is a bit of irony in this story. From an island you can visit only by ferry, we are unlocking the mysteries of a gene critical to ferrying cholestrol out of the body....
*Accommodations on the* *Western Shore* *of the Chesapeake Bay* *Virginia's Northern Neck*	Tangier Disease is an inherited blood disorder involving decreased concentrations of fat compounds in the blood called high-density lipoproteins (HDL) (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. Other Names Alpha High-Density Lipoprotein Deficieny Alphalipoproteinemia Analphalipoproteinemia Familial Alpha-Lipoprotein Deficiency Familial High-Density Lipoprotein Deficiency High density lipoproteindeficiency, type 1 (HDLDT1) Tangier Disease Neuropathy Epidemiology Prevalence Documentation shows that as of 1988, 27 cases of Tangier Disease had been reported and in 1992 the reported cases were still fewer than 50 persons worldwide. The majority of the cases tend to localize in one single area of the U.S., Tangier Island, Virginia. The fact that most of the people that are affected by Tangier disease all live in close proximity to one another could be due to Founder's effect. The original settlers to the island came in 1686 and it is possible that one or two of them were carriers of the disease or actually had the symptoms and passed it down through the blood line. Signs and Symptoms Characteristics of Tangier Disease include increased levels or even a complete absence of high-density lipoproteins (HDL) concentrations in one's plasma, low cholesterol levels in the plasma, increased cholesteryl esters in the tonsils, spleen, liver, skin and lymph nodes. One easily visual characteristic usually found in children with Tangier disease is the presence of enlarged, yellow-orange tonsils. Research Recent discoveries Initial research of Tangier disease showed a marked decrease in the HDL concentrations when compared to normal controls. In some cases the reduction was as great as 50%. Scientists studied the HDL concentrations and looked for any possible links in its involvement with the disease. They specifically looked at the apo A-I (apolipoprotein) concentrations, which is a major protein component of HDL. The main hypothesis was that apo A-I was structurally abnormal. Studies proved that this was incorrect because the DNA- derived protein sequence for Tangier apo A-I was identical to the control groups apo A-I sequence. Causes Scientists discovered that the cause of Tangier disease is involved with the intracellular membrane trafficking of the HDL. Normally macrophages inside the cell have receptors that bind the HDL. After the HDL is bound it is transported into the endosomes. The endosome is transported through the cell without any degradation by the lysosome and the HDL is eventually resecreted from the cell. It is during this cycle that there are problems for the Tangier disease people. When the HDL is allowed to bind to the receptor monocyte, the two stick together but they never separate. The HDL is not resecreted outside the cell. The data suggest that there is a deficiency in the cellular metabolism of HDL in the Tangier monocytes. The HDL-monocyte unit together also supports the observed condition of high concentrations of excess cholesterol in body tissues. Treatment Currently the treatment for Tangier patients is dependent on the various symptoms, ranging from heart surgery to removal of organs. Gene therapy has been proposed as a possible treatment but is difficult because there isn't anything wrong specifically with the gene involved in the HDL conversion. The problem is in the cellular transportation. Many of the specific processes within the cell are still not known so any extensive treatment is still investigational. Above: Excerpts from: http://wiki.medpedia.com/Tangier_Disease
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