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Acid sphingomyelinase inhibition improves motor behavioral deficits and neuronal loss in an amyotrophic lateral sclerosis mouse model
Byung Jo Choi1,2,# (Graduate student), Kang Ho Park1,3,# (Graduate student), Min Hee Park1,3 (Research Professor), Eric Huang4 (Professor), Seung Hyun Kim5 (Professor), Jae-sung Bae1,3 (Professor), Hee Kyung Jin1,2,* (Professor)
1KNU Alzheimer’s disease Research Institute, Kyungpook National University,
2Department of Laboratory Animal Medicine, College of Veterinary Medicine, Kyungpook National University,
3Department of Physiology, School of Medicine, Kyungpook National University,
4Department of Pathology, University of California San Francisco,
5Department of Neurology, Hanyang University College of Medicine
Amyotrophic lateral sclerosis (ALS) is an incurable neurodegenerative disease characterized by the degeneration of motor neurons in the spinal cord. Main symptoms are manifested as weakness, muscle loss, and muscle atrophy. Some studies have reported that alterations in sphingolipid metabolism may be intimately related to neurodegenerative diseases, including ALS. Acid sphingomyelinase (ASM), a sphingolipid-metabolizing enzyme, is considered an important mediator of neurodegenerative diseases. Herein, we show that ASM activity increases in samples from patients with ALS and in a mouse model. Moreover, genetic inhibition of ASM improves motor function impairment and spinal neuronal loss in an ALS mouse model. Therefore, these results suggest the role of ASM as a potentially effective target and ASM inhibition may be a possible therapeutic approach for ALS.
Abstract, Accepted Manuscript(in press) [Submitted on September 13, 2022, Accepted on October 7, 2022]
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