Immunological aspects of immunodeficiency diseases part 92

Immune 3

 

IMMUNE DYSREGULATION POLYENDOCRINOPATHY AND ENTEROPATHY, X-LINKED SYNDROME

The product of the FOXP3 gene (belonging to the forkhead family) is required for the generation and the function of CD4 CD25 T-regulatory cells. Mice that are FOXP3-/- lack these cells and develop a syndrome characterized by  lymphoproliferation and autoimmunity. Expression of ectopic FOXP3 confers suppressive function on CD4 and CD25 T cells. Human males with mutations of the FOXP3 gene (which is located on the X chromosome) develop a syndrome characterized by neonatal endocrinopathies (including type 1 diabetes) enteropathy, eczema, immune thrombocytopenia, and cachexia. In these patients, pancreatic islet cell and intestinal mucosal damage are secondary to infiltration  of the tissues with mononuclear cells and plasma cells, signifying an autoimmune pathogenesis. This condition is called the immune dysregulation polyendocinopathy and enteropathy, X-linked (IPEX) syndrome. This condition provides evidence supporting current concepts on the role of T-regulatory cells in preventing autoimmunity.

Advertisements

About azaleaazelia

A nice person... :)
This entry was posted in Tak Berkategori. Bookmark the permalink.

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out /  Change )

Google photo

You are commenting using your Google account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )

Connecting to %s