Implication of the inflammasome protein complex in the autoimmune lymphoproliferative syndrome (ALPS)

Tali Finger

Vol. 1 Issue 2. p. 1-10 (2019)

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ABSTRACT: Autoimmune diseases are chronic diseases that affect 5% to 8% of the world’s population. Exploring, in-depth, a specific autoimmune disease such as the autoimmune lymphoproliferative syndrome (ALPS) may help broaden our knowledge of all forms of autoimmune diseases. Our research project consisted of two parts. We first reported a case of a patient with a possible autoimmune disorder in the spectrum of ALPS-like disorders. We then suggested the implication of a protein complex called the Inflammasome in ALPS and investigated the yet unclear interaction between innate immunity and cellular apoptosis. We used systems biology tools, such as Ingenuity® and Cytoscape®. These analyses showed that: 1) the studied patient report fits the ALPS spectrum, which is, therefore, significantly broader than that described in the literature; 2) the Inflammasome is indeed implicated in the reported case; and 3) ALPS is not only involved in the organism’s adaptive immunity as it was believed but is also in the organism’s innate immunity; 4) the relationship between innate immunity and cellular apoptosis is broader than previously thought. The study’s findings provide new insights on these disorders and may help elucidate mechanisms implicated in autoimmune diseases, and thus contribute to the development of more effective treatments.

KEYWORDS: Immunology; Innate; Immunity; Apoptosis; ALPS; Inflammasome