Blau Syndrome

Combinations of PYD, CARD, NACHT, and or LRR domains are found in over 20 other proteins encoded in the human genome (Tschopp et al. 2003 Ting and Davis 2004 Martinon and Tschopp 2005), many of which are expressed in immune tissues. One such molecule is the NOD2 CARD15 protein, which contains two N-terminal CARD domains, followed by a NACHT domain and ten LRR motifs (Fig. 4). Mutations in the LRR of NOD2 CARD15 were first associated with Crohn's disease, one of two common forms of inflammatory...

Immunological Memory and Chronic Autoimmune Diseases

Though most autoimmune diseases become chronic, spontaneous remission occurs in a small, but considerable proportion of patients suffering from various autoimmune diseases 18, 19 . Complete remission is common among autoimmune diseases that had been induced by an exogenous stimulus in individuals bearing an otherwise intact immune system. This applies for most animal models, where autoreactivity is induced in animals that otherwise would not develop that autoimmune disorder such as experimental...

References

Boumpas DT, Austin HA 3rd, Fessler BJ, Balow JE, Klippel JH, Lockshin MD (1995) Systemic lupus erythematosus emerging concepts. Part 1. Renal, neuropsychiatry cardiovascular, pulmonary, and hematologic disease. Ann Intern Med 122 940-950 2. Arbuckle MR, McClain MT, Rubertone MV, Scofield RH, Dennis GJ, James JA, Harley JB (2003) Development of autoantibodies before the clinical onset of systemic lupus erythematosus. N Engl J Med 349 1526-1533 3. Wakeland EK, Liu K, Graham RR, Behrens TW (2001)...

Rationales of B Cell Targeted Therapy in Autoimmune Diseases

Although identification of the central antigens in a number of rheumatic diseases is still lacking, formation of T cell B cell aggregates and the formation of germinal centre-like structures can be correlated with increasing disease activity, including the production of autoantibodies (Leadbetter et al. 2002 Shlomchik et al. 2001). It needs to be shown whether apparent differences in the underlying pathologic process, with the disease activity likely escalating from 1) non-organized infiltrate...

Syndrome of Pyogenic Arthritis Pyoderma Gangrenosum and Acne

As noted above, the B-box and coiled-coil motifs of pyrin are also protein interaction domains, mediating binding to proline-serine-threonine phos-phatase interacting protein 1 (PSTPIP1) (Shoham et al. 2003), also known as CD2-binding protein 1 (CD2BP1) (Li et al. 1998). PSTPIP1 is a 416-amino acid protein encoded on chromosome 15q24 (Wise et al. 2002). PSTPIP1 consists of an N-terminal Fer-CIP4 domain (Aspenstrom 1997), followed by a coiled-coil region, and finally an SH3 domain at the...