How many people have IRAK-4?

IRAK-4 deficiency is a very rare condition, although the exact prevalence is unknown. At least 49 individuals with this condition have been described in the scientific literature.

What causes IRAK4 deficiency?

The deficiency is caused by mutations in the IRAK4 gene and is inherited in an autosomal recessive pattern. Treatment may include intravenous immunoglobulin therapy (IVIG), taking antibiotics before an infection develops, and vaccines.

What does Irak-4 do?

The IRAK-4 protein is part of a signaling pathway that is involved in early recognition of pathogens and the initiation of inflammation to fight infection. In particular, the IRAK-4 protein relays signals from proteins called Toll-like receptors and IL-1 receptor-related proteins.

What is Irak immunology?

Interleukin-1 receptor-associated kinases (IRAK) play a central role in inflammatory responses by regulating the expression of various inflammatory genes in immune cells. These signals are critical for elimination of viruses, bacteria, and cancer cells, as well as for wound healing.

How do you spell Iraq plural?

Iraqi or Iraqis (in plural) means from Iraq, a country in the Middle East, and may refer to:

  1. Iraqi people or Iraqis, people from Iraq or of Iraqi descent.
  2. A citizen of Iraq, see demographics of Iraq.

What is MyD88 deficiency?

MyD88 deficiency is an inherited disorder of the immune system (primary immunodeficiency). This primary immunodeficiency affects the innate immune response, which is the body’s early, nonspecific response to foreign invaders (pathogens).

What is irak4 inhibitor?

Interleukin-1 receptor-associated kinase 4 (IRAK-4) inhibitors are novel agents designed to suppress immune signaling pathways involved in inflammation and joint destruction in RA. Four IRAK-4 inhibitors have entered clinical trials.

What is the Myddosome?

The myddosome is one of several newly- defined receptor-proximal complexes that control innate immune signal transduction. Analogous complexes operate to control inflammasome assembly and the RIG-I and TNF receptor signaling pathways.

What is pyogenic infections?

Infections in which pus is produced are known as pyogenic, that is, pus-producing infections. A wound, whether surgical or accidental, has a tendency to become infected. Strains of Staphylococcus aureus, which are resistant to many of the available antibiotics, are not uncommon in hospitals.

What is MYD88 mutation?

MYD88 mutation is the most common genetic abnormality in the activated B-cell-like (ABC) subtype of diffuse large B-cell lymphoma (DLBCL), detected in 40% of cases. Mutations are rare in the germinal center B-cell-like (GCB) subtype, so mutation analysis can be useful to differentiate between the ABC and GCB subtypes.