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KMID : 0624620220550120621
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BMB Reports
2022 Volume.55 No. 12 p.621 ~ p.626
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Acid sphingomyelinase inhibition improves motor behavioral deficits and neuronal loss in an amyotrophic lateral sclerosis mouse model
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Choi Byung-Jo
Park Kang-Ho
Park Min-Hee
Huang Eric Jinsheng
Kim Seung-Hyun
Bae Jae-Sung
Jin Hee-Kyung
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Abstract
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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.
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KEYWORD
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Acid sphingomyelinase, Amyotrophic lateral sclerosis, FUS, Motor behavioral dysfunction, Motor neuronal loss
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