Autoimmunity in the Pathology of Amyotrophic Lateral Sclerosis

MARIO RAFAEL PAGANI, LAURA ELISABETH GONZALEZ AND OSVALDO DANIEL UCHITEL

Frontier in Bioscience

Frontier in Bioscience

Lugar: Albertson, NY; Año: 2009

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease affecting particularly motor neurons for which no cure or treatment is available. Although the cause of ALS remains unknown, accumulative evidence suggests an autoimmune mechanism of pathogenesis. In this review we summarize the current evidence related to autoimmunity in the sporadic form of ALS and discuss the potential underlying pathogenic mechanisms and perspectives. Presented evidence supports the view that humoral immune responses against motor nerve terminals can initiate a series of physiological changes leading to alteration of calcium homeostasis. In turn, loss of calcium homeostasis may induce neuronal death through apoptotic signaling pathways. Additional approaches identifying specific molecular features of this hypothesis are required, which will hopefully allow us to develop techniques of early diagnosis and effective therapies.