Myeloid differentiation primary response protein MyD88

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Common Name:Myeloid differentiation primary response protein MyD88
Other Names, such as IUPAC Name
Myeloid differentiation marker MyD88, MyD marker MyD88
Biochemical Information
Molecular Structure
Important Issues in Man
Relevant Clinical Literature
UK Guidance
Other Wikis

The MYD88 gene (myeloid differentiation primary response gene 88) at 3p22.2 codes for the 296 amino acid pro-peptide of myeloid differentiation primary response protein MyD88, a key effector of innate immunity. As well as homodimers it forms heterodimers with the TIR domain-containing adapter protein (Toll/interleukin-1 receptor domain-containing adapter protein). There are 6 isoforms.

Myeloid differentiation primary response protein MyD88 is an adapter protein that forms dimers involved in the Toll-like receptor and IL-1 receptor signalling pathway in the innate immune response leading to NF-kappa-B activation, cytokine secretion and the inflammatory response. MyD88 links IL-1 receptor or TLR family members with a C-terminal TIR (Toll IL-1R) domain interaction. The N-terminal death domain (DD) associates with IL-1R-associated kinase (IRAK) family kinases via homotypic protein-protein interaction. A complex is formed called the myddosome made up of a ring of six MyD88 and four IRAK2 DDs in a helix. MyD88-mediated signalling in intestinal epithelium maintains gut homeostasis and controls the expression of the antimicrobial lectin regenerating islet-derived protein 3-gamma.

Defects cause life-threatening, often recurrent pyogenic bacterial infections, including invasive pneumococcal disease. It also protects against endotoxic shock, so the death may not be rapid ! The gene is highly conserved in animals.The MYD88 mutation L265P is very common in Waldenstrom's macroglobulinaemia.