S100A6 overexpression within astrocytes associated with impaired axons from both ALS mouse model and human patients.

摘要: Astrogliosis is one of the earliest pathological changes observed in neurodegenerative diseases general and amyotrophic lateral sclerosis (ALS) particular. ALS characterized by selective degeneration motoneurons. There are 2 forms disease: sporadic (SALS), comprising 90%-95% cases, familial (FALS), 5%-10% cases. FALS an age-dependent autosomal dominant disorder which mutations homodimeric enzyme Cu/ Zn superoxide dismutase 1 (SOD1) linked to disease. The animal model for this disease a transgenic mouse expressing mutated human SOD1(G93A) gene. Here we show immunohistochemistry double immunofluorescence that astrocytes located near impaired axons motoneurons were selectively programmed die overexpressed S100A6, Ca2+/Zn2+ binding protein able translocate into nucleus. Transgenic mice overexpressing SOD1 gene patients suffering from SALS showed astrocytic S100A6 expression. For instance, pyramidal tract could be macroscopically detected on S100A6-labeled spinal cord brainstem sections patients. non-mutated did not overexpress although glial fibrillary associated astrogliosis was seen. Although these results do give any clue about beneficial or detrimental role played its induction may assumed appropriately serve some function(s).