Table of Contents > Allergies > Toll-like receptors Print

Toll-like receptors

Image

Also listed as: WAS
Related terms
Background
Author information
Bibliography
Functions

Related Terms
  • Antibodies, auto recessive, B-cells, bone marrow, bone marrow transplant, CBC, genetic disorder, immune system, immunodeficiency, inherited disorder, inherited immunodeficiency, leukocytes, leukemia, lymphoma, lymphocytes, malignancy, platelets, pneumonia, red blood cells, T-cells, thrombocytes, thrombocytopenia, tumor, WASP, white blood cells, Wiskott Aldrich syndrome, Wiskott-Aldrich syndrome protein, X-linked.

Background
  • Wiskott-Aldrich syndrome (WAS) is an inherited, immunodeficiency disorder that occurs almost exclusively in males. The recessive genetic disorder is caused by a mutation in the WAS (Wiskott-Aldrich syndrome) gene, which is an X-linked trait. The gene mutation leads to abnormalities in B- and T-lymphocytes (white blood cells), as well as blood platelet cells. In a healthy individual, the T-cells provide protection against viral and fungal infection, the B cells produce antibodies, and platelets are responsible for blood clotting to prevent blood loss after a blood vessel injury.
  • Individuals diagnosed with WAS suffer from recurrent infections, eczema and thrombocytopenia (low levels of platelets).
  • Before 1935, patients only lived an average of eight months. Today, patients usually live an average of eight years, according to a recent case study. The cause of death is usually attributed to extensive blood loss. However, cancer (especially leukemia) is common and often fatal among WAS patients.
  • The only possible cure for WAS is a bone marrow transplant. However, if a patient's family member is not a possible match for a bone marrow donation, patients may have to wait years for a potential donor. Other aggressive treatments may also increase a patient's life expectancy. For instance, one study found that patients who underwent splenectomy (removal of the spleen) lived to be more than 25 years old. The spleen may harbor too many platelets, and cause a decrease in the number of platelets in circulation. Antibiotics, antivirals, antifungals, chemotherapeutic agents, immunoglobulins and corticosteroids have also been used to relieve symptoms and treat infections and cancer associated with WAS.
  • Researchers estimate that about four people per one million live male births develop the disease in the United States.
  • The syndrome is named after Dr. Robert Anderson Aldrich, an American pediatrician who described the disease in a family of Dutch-Americans in 1954, and Dr Alfred Wiskott, a German pediatrician who discovered the syndrome in 1937. Wiskott described three brothers with a similar disease, whose sisters were unaffected.

Author information
  • This information has been edited and peer-reviewed by contributors to the Natural Standard Research Collaboration (www.naturalstandard.com).

Bibliography
  1. Binder V, Albert MH, Kabus M, et al. The genotype of the original Wiskott phenotype. N Engl J Med. 2006 Oct 26;355(17):1790-3.
  2. Jin Y, Mazza C, Christie JR, et al. Mutations of the Wiskott-Aldrich Syndrome Protein (WASP): hotspots, effect on transcription, and translation and phenotype/genotype correlation. Blood. 2004 Dec 15;104(13):4010-9. Epub 2004 Jul 29.
  3. Natural Standard: The Authority on Integrative Medicine. .
  4. St. Jude Children's Research Hospital. Inherited Immunodeficiencies: Wiskott-Aldrich Syndrome (WAS). .
  5. U.S. Immune Deficiency Foundation. The Wiskott Aldrich Syndrome. .

Functions
  • Toll-like receptors are an important part of the body's innate immune system, also called the natural immune system. These receptors are part of the body's first line of defense against harmful substances that enter the body.
  • The receptors, located on the outside of immune system cells, are constantly surveying surrounding cells and tissues for pathogens. Each type of TLR is able to recognize a wide range of pathogens. This is because TLRs recognize pathogens based on their pathogen-associated molecular pattern (PAMP). Since large groups of microbes share the same PAMPs, a single TLR is able to recognize many different invaders.
  • Toll-like receptors (TLRs) become activated once they identify and bind to a foreign substances that enter the body. Once stimulated, the TLRs activate signaling pathways that trigger an immune response. This immune response eventually leads to the expression of many antimicrobial genes that are necessary for immune cells to destroy the invading substances. TLRs also trigger the release of inflammatory chemicals called cytokines.
  • In 1997, researchers discovered that a specific TLR, called TLR4, can activate certain genes needed to initiate an adaptive immune response. Adaptive immunity is a type of protection that develops over the course of an individual's life. Adaptive immunity involves the development of immunoglobulin antibodies that respond to specific foreign substances that enter the body. When individuals are exposed to certain foreign invaders, they develop antibodies against the pathogens. Then, if the same substance enters the body in the future, the body is then prepared to respond quickly because the antibodies are already developed. The antibodies detect and bind to foreign substances that enter the body signaling other immune cells to destroy it. However, it remains unclear exactly how TLR4 is involved in adaptive immunity.

Copyright © 2011 Natural Standard (www.naturalstandard.com)


The information in this monograph is intended for informational purposes only, and is meant to help users better understand health concerns. Information is based on review of scientific research data, historical practice patterns, and clinical experience. This information should not be interpreted as specific medical advice. Users should consult with a qualified healthcare provider for specific questions regarding therapies, diagnosis and/or health conditions, prior to making therapeutic decisions.

62 Marshall St
Rochester, NY 14607
585-454-2667
585-454-0343 (fax)
Email Our Store
Driving Directions

  STORE HOURS
 Mon8:00am-8:00pm
 Tue8:00am-8:00pm
 Wed8:00am-8:00pm
 Thu8:00am-8:00pm
 Fri8:00am-8:00pm
 Sat9:00am-7:00pm
 Sun10:00am-7:00pm
 

Co-op Connections

Follow us on Instagram!

Follow us on Twitter!

Join our Facebook community!

Email newsletter sign up

Email Newsletter Archive
Click here>>

Download The Rutabaga Rap

Rutabaga Rap Archive
Click here>>

About Our Co-op

Current Job Openings
See current openings>>

Top 10 Reasons for Shopping
Learn more>>

Why become an owner?
Learn why>>

Owner Application
Download (pdf)>>

All About Co-ops
Learn more>>

Our Board of Directors
Learn more>>

Global Ends Policy
Learn more>>

Bylaws
Learn more>>

Advertise in Our Newsletter
Learn more>>

Job Application
Download (pdf)>>

Discover your local co-op!