MicroRNA expression in animal models of amyotrophic lateral sclerosis and potential therapeutic approaches

Martinez, Bridget; Peplow, Philip V.

doi:10.4103/1673-5374.322431

MicroRNA expression in animal models of amyotrophic lateral sclerosis and potential therapeutic approaches

Journal Article · Fri Apr 01 00:00:00 EDT 2022 · Neural Regeneration Research

DOI:https://doi.org/10.4103/1673-5374.322431· OSTI ID:2470445

Martinez, Bridget ^[1]; Peplow, Philip V. ^[2]

Los Alamos National Laboratory (LANL), Los Alamos, NM (United States); St. Georges University School of Medicine (Grenada)
University of Otago, Dunedin (New Zealand)

A review of recent animal models of amyotrophic lateral sclerosis showed a large number of miRNAs had altered levels of expression in the brain and spinal cord, motor neurons of spinal cord and brainstem, and hypoglossal, facial, and red motor nuclei and were mostly upregulated. Among the miRNAs found to be upregulated in two of the studies were miR-21, miR-155, miR-125b, miR-146a, miR-124, miR-9, and miR-19b, while those downregulated in two of the studies included miR-146a, miR-29, miR-9, and miR-125b. A change of direction in miRNA expression occurred in some tissues when compared (e.g., miR-29b-3p in cerebellum and spinal cord of wobbler mice at 40 days), or at different disease stages (e.g., miR-200a in spinal cord of SOD1(G93A) mice at 95 days vs. 108 and 112 days). In the animal models, suppression of miR-129-5p resulted in increased lifespan, improved muscle strength, reduced neuromuscular junction degeneration, and tended to improve motor neuron survival in the SOD1(G93A) mouse model. Suppression of miR-155 was also associated with increased lifespan, while lowering of miR-29a tended to improve lifespan in males and increase muscle strength in SOD1(G93A) mice. Overexpression of members of miR-17~92 cluster improved motor neuron survival in SOD1(G93A) mice. Treatment with an artificial miRNA designed to target hSOD1 increased lifespan and improved muscle strength in SOD1(G93A) animals. Further studies with animal models of amyotrophic lateral sclerosis are warranted to validate these findings and identify specific miRNAs whose suppression or directed against hSOD1 results in increased lifespan, improved muscle strength, reduced neuromuscular junction degeneration, and improved motor neuron survival in SOD1(G93A) animals.

View Accepted Manuscript (DOE)

Research Organization:: Los Alamos National Laboratory (LANL), Los Alamos, NM (United States)

Sponsoring Organization:: USDOE National Nuclear Security Administration (NNSA)

Grant/Contract Number:: 89233218CNA000001

OSTI ID:: 2470445

Journal Information:: Neural Regeneration Research, Journal Name: Neural Regeneration Research Journal Issue: 4 Vol. 17; ISSN 1673-5374

Publisher:: Chinese Association of Rehabilitation Medicine (CARM)Copyright Statement

Country of Publication:: United States

Language:: English

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59 BASIC BIOLOGICAL SCIENCES
amyotrophic lateral sclerosis
animal model
brain
brainstem
cell biology
microRNA
motor neuron degeneration
neurosciences & neurology
spinal cord
therapeutic approaches

MicroRNA expression in animal models of amyotrophic lateral sclerosis and potential therapeutic approaches

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