Elsevier

Autoimmunity Reviews

Volume 8, Issue 1, October 2008, Pages 5-8
Autoimmunity Reviews

Clearance deficiency—A potential link between infections and autoimmunity

https://doi.org/10.1016/j.autrev.2008.07.049Get rights and content

Abstract

Cell death plays a pivotal role in development and homeostasis of multicellular organisms. Apoptosis represents the physiological and anti-inflammatory form of cell death. Advanced apoptosis and necrosis are rather pro-inflammatory. Several “find-me”- and “eat-me”-signals support the “swift and silent” removal of dying cells. If the highly controlled process of dying cell removal fails, they may progress to secondary necrosis and provoke autoimmunity. There are several reports describing clearance deficiency as a possible mechanism in the etiopathogenesis of SLE. Under certain conditions, increased phagocytosis of nuclear material may be found in a subgroup of patients with SLE. Complement proteins and autoantibodies may modify engulfment of apoptotic remnants and shift the clearance process towards inflammation. Taken together, clearance deficiency leads to the accumulation of apoptotic remnants and breaking of tolerance to self. Besides, enhanced uptake of nuclear immune complexes may maintain chronic autoimmunity in patients with SLE.

Section snippets

Apoptosis and the progression to secondary necrosis

Apoptosis—the programmed and natural form of cell death—plays a pivotal role in embryogenesis, development and homeostasis of multicellular organisms. In each moment new cells develop and at the same time other cells must die. After cell shrinkage, chromatin condensation, and DNA cleavage, cellular material gets packed into membrane-derived apoptotic bodies or blebs. These are usually rapidly and efficiently engulfed by neighbouring cells or macrophages in tissues. Notably, during this highly

Molecules involved in phagocytosis of dying cells

Dying cells make use of “find-me”- and “eat-me”-signals for an efficient recognition and engulfment by phagocytes. One “find-me”-signal secreted by apoptotic cells is the soluble lysophosphatidylcholine (LPC) [4]. Exposed and oxidised phosphatidylserine (PS) represents an early “eat-me”-signal. Phagocyte receptors such as CD36, oxLDL receptor, and Mer Kinase bind to PS via several bridging molecules like growth arrest-specific gene 6 (Gas6), milk fat globule EGF/factor VIII (MFG-E8),

Clearance deficiencies, inflammation, and SLE

If invading pathogens are not eliminated or cleared they may cause infection and inflammation. Similarly, if clearance of apoptotic cells is deficient, dying cells will progress to secondary necrosis and leak modified autoantigens, putative “non-self” material, and danger signals. Inflammatory responses or autoimmune reactions as well as chronic autoimmune diseases such as SLE may be the consequences.

SLE is a multifactorial systemic disease. Environmental factors (such as UV radiation or viral

Take-home messages

  • Apoptosing cells are cleared in an efficient and silent manner by phagocytes with the support of “find-me”- and “eat-me”-signals.

  • The alteration of the glycosylation pattern during apoptosis may serve as “late back-up-eat-me signal”.

  • The altered surface of late apoptotic cells results from a substitution of internal ER-membranes after the process of blebbing.

  • Clearance deficiency may lead to the accumulation of nuclear material and may thus provoke autoimmunity, as in SLE disease.

  • Clearance may

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  • Cited by (0)

    Grant support: This work was supported by “Deutsche Forschungsgemeinschaft” SFB 643 (project B5), by the European Commissions [E.U. (QLK3-CT-2002-02017_APOCLEAR)], by the Lupus Erythemathodes Selbsthilfegemeinschaft e.V., by the Programme Alban, the European Union Program of High Level Scholarships for Latin America, scholarships no. E04D047956VE to L. E. M. and E07D400430VE to R.A.C. Part of this work was funded by an intramural grant from the ELAN fond and the Training Grant GK592 from the German Research Community (DFG) to C.S and K.S.

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