Abstract
Amyotrophic lateral sclerosis is a late-onset disorder primarily affecting motor neurons and leading to progressive and lethal skeletal muscle atrophy. Small RNAs, including microRNAs (miRNAs), can serve as important regulators of gene expression and can act both globally and in a tissue-/cell-type-specific manner. In muscle, miRNAs called myomiRs govern important processes and are deregulated in various disorders. Several myomiRs have shown promise for therapeutic use in cellular and animal models of ALS; however, the exact miRNA species differentially expressed in muscle tissue of ALS patients remain unknown. Following small RNA-Seq, we compared the expression of small RNAs in muscle tissue of ALS patients and healthy age-matched controls. The identified snoRNAs, mtRNAs and other small RNAs provide possible molecular links between insulin signaling and ALS. Furthermore, the identified miRNAs are predicted to target proteins that are involved in both normal processes and various muscle disorders and indicate muscle tissue is undergoing active reinnervation/compensatory attempts thus providing targets for further research and therapy development in ALS.
Highlights
MicroRNAs and other small RNAs in muscle tissue of patients with Amyotrophic lateral sclerosis (ALS) and healthy age-matched controls
We obtained muscle biopsies from 12 patients with ALS and 11 control subjects, due to low RNA integrity number (RIN) one patient sample was excluded from the analysis
The ALS FRS score of patients ranged from 18 to 43 (average 28.3 (±6.9)), only patients who could walk were included in the study as we assumed that the severity of muscle atrophy in immobile patients would not allow for adequate comparison of muscle tissue
Summary
MicroRNAs and other small RNAs in muscle tissue of patients with ALS and healthy age-matched controls. Amyotrophic lateral sclerosis is a late-onset disorder primarily affecting motor neurons and leading to progressive and lethal skeletal muscle atrophy. Following small RNA-Seq, we compared the expression of small RNAs in muscle tissue of ALS patients and healthy age-matched controls. The identified miRNAs are predicted to target proteins that are involved in both normal processes and various muscle disorders and indicate muscle tissue is undergoing active reinnervation/compensatory attempts providing targets for further research and therapy development in ALS. Amyotrophic lateral sclerosis (ALS) is a late-onset disorder primarily affecting upper and lower motor neurons leading to progressive and severe skeletal muscle atrophy. As well as giving rise to exotic DNA features such as G-quadruplexes and i-motifs[17,18], the expanded repeats undergo both aberrant and unconventional processing (reviewed in Vatovec et al.19), which further supports disease-associated changes in RNA metabolism as a core mechanism in ALS pathogenesis
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