What is the function of Birbeck granules?

Function. The function of Birbeck granules is debated, but one theory is that they migrate to the periphery of the Langerhans cells and release their contents into the extracellular matrix. Another theory is that the Birbeck granule functions in receptor-mediated endocytosis, similar to clathrin-coated pits.

What are Birbeck granules?

Birbeck granules are unusual rod-shaped structures specific to epidermal Langerhans cells, whose origin and function remain undetermined. In the steady state, Langerin is predominantly found in the endosomal recycling compartment and in Birbeck granules.

What is Langerhans Cell Histiocytosis X?

Langerhans cell histiocytosis (also called histiocytosis X) is a rare disorder that primarily affects children. This disorder is characterized by an abnormal increase in certain immune cells called histiocyte cells.

Is histiocytosis X genetic?

Langerhans cell histiocytosis is a rare disorder that can affect people of all ages. The highest rate is among children ages 5 to 10. Some forms of the disorder are genetic, which means they are inherited.

What is LCH disease?

Langerhans cell histiocytosis (LCH) is a rare disease that begins in LCH cells. LCH cells are a type of dendritic cell that normally helps the body fight infection. Sometimes mutations (changes) develop in genes that control how dendritic cells function. These include mutations of the BRAF, MAP2K1, RAS, and ARAF genes.

Does histiocytosis go away?

In many people with Langerhans cell histiocytosis, the disorder eventually goes away with appropriate treatment. It may even disappear on its own, especially if the disease occurs only in the skin.

Can LCH be cured?

Is there a cure for LCH? While some patients go into remission and may live normal lives with or without treatment, we usually don’t use the term “cure” with this disease. No specific amount of time without active disease has yet been established for adults to determine when a patient is considered to be cured.

Where are Birbeck granules found in the body?

Birbeck granules, also known as Birbeck bodies, are rod shaped or “tennis-racket” cytoplasmic organelles with a central linear density and a striated appearance. First described in 1961 (where they were simply termed “characteristic granules”), they are solely found in Langerhans cells.

What kind of immunostain is used for Birbeck granules?

The use of Langerin immunostain has largely supplanted the need for ultrastructural analysis. The Birbeck granule is a rod-shaped bilaminar disk with an internal zipper-like pattern of striations, often with a bulbous dilatation at one end, like a tennis racquet.