Abstract
Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease that causes compromised function of motor neurons and neuronal death. However, oculomotor neurons are largely spared from disease symptoms. The underlying causes for sporadic ALS as well as for the resistance of oculomotor neurons to disease symptoms remain poorly understood. In this bioinformatic-analysis, we compared the gene expression profiles of spinal and oculomotor tissue samples from control individuals and sporadic ALS patients. We show that the genes GAD2 and GABRE (involved in GABA signaling), and CALB1 (involved in intracellular Ca2+ ion buffering) are downregulated in the spinal tissues of ALS patients, but their endogenous levels are higher in oculomotor tissues relative to the spinal tissues. Our results suggest that the downregulation of these genes and processes in spinal tissues are related to sporadic ALS disease progression and their upregulation in oculomotor neurons confer upon them resistance to ALS symptoms. These results build upon prevailing models of excitotoxicity that are relevant to sporadic ALS disease progression and point out unique opportunities for better understanding the progression of neurodegenerative properties associated with sporadic ALS.
Highlights
Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease that causes death and the compromised function of motor neurons
Our results suggest that the downregulation of these genes and processes in spinal tissues are related to sporadic ALS disease progression and their upregulation in oculomotor neurons confer upon them resistance to ALS symptoms
CALB1 are downregulated in the spinalin sporadic ALS patients and show that the genes GAD2, GABRE, and CALB1 are downregulated tissues of sporadic patients ALS
Summary
Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease that causes death and the compromised function of motor neurons. The progression of the disease has several complications, including increased muscle weakness and loss of coordination [1]. There are two types of ALS: sporadic (95% of diagnosed cases) and familial (5% of diagnosed cases). While there is considerable overlap among the genes that are associated with both forms of ALS, the underlying causes for sporadic ALS are less clear [5]. Critical evaluations of human gene expression datasets continue to remain a valuable approach for identifying the underlying causes of this disease
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