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Molecular Abnormalities in Sporadic Amyotrophic Lateral Sclerosis (ALS)
Author(s) -
Perrin Hannah,
Ding Fei Fei,
Segalia Emily,
Vonsattel Jean Paul,
Monte Suzanne
Publication year - 2007
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.21.5.a22-a
Subject(s) - amyotrophic lateral sclerosis , sod1 , sod2 , pathogenesis , biology , motor neuron , receptor , superoxide dismutase , immunology , spinal cord , neuroscience , disease , medicine , pathology , genetics , endocrinology , oxidative stress
ALS is a neurodegenerative disease affecting both upper and lower motor neurons. A mutation in the superoxide dismutase I (SOD1) gene has been implicated in the familial form of disease; however, little is known about the pathogenesis of sporadic ALS, which accounts for 90–95% of the cases. In addition to functional impairment of SOD1 or other SOD genes, the consistent distribution of neurodegenerative lesions in primary motor neurons raises question about the potential role of poliovirus receptor (CD155) and its accessory proteins in the pathogenesis of ALS. This study examines the expression levels of SOD1, SOD2, CD155, CD 112 (transmembrane molecule related to CD155 and named Poliovirus Receptor Related2), and CD 44 (blocks binding to CD155) in spinal cord tissue from ALS and age‐matched control cases using quantitative RT‐PCR. ALS samples had significantly reduced levels of CD155 and SOD1, and increased expression of SOD2 relative to control. The results suggest that sporadic ALS is mediated by motor neuron specific impairment of poliovirus receptor function, together with SOD1 deficiency. Up‐regulation of SOD2 may represent a compensatory response to preserve neuronal viability.