首页|REEP1 Preserves Motor Function in SOD1G93A Mice by Improving Mitochondrial Function via Interaction with NDUFA4
REEP1 Preserves Motor Function in SOD1G93A Mice by Improving Mitochondrial Function via Interaction with NDUFA4
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A decline in the activities of oxidative phos-phorylation(OXPHOS)complexes has been consistently reported in amyotrophic lateral sclerosis(ALS)patients and animal models of ALS,although the underlying molecular mechanisms are still elusive.Here,we report that receptor expression enhancing protein 1(REEP1)acts as an impor-tant regulator of complex Ⅳ assembly,which is pivotal to preserving motor neurons in SOD1G93A mice.We found the expression of REEP1 was greatly reduced in transgenic SOD1G93A mice with ALS.Moreover,forced expression of REEP1 in the spinal cord extended the lifespan,decelerated symptom progression,and improved the motor performance of SOD1G93A mice.The neuromuscular synaptic loss,glio-sis,and even motor neuron loss in SOD1G93A mice were alleviated by increased REEP1 through augmentation of mitochondrial function.Mechanistically,REEP1 associates with NDUFA4,and plays an important role in preserving the integrity of mitochondrial complex Ⅳ.Our findings offer insights into the pathogenic mechanism of REEP1 deficiency in neurodegenerative diseases and suggest a new therapeutic target for ALS.
Department of Cell Biology,Shandong Provincial Key Laboratory of Mental Disorders,School of Basic Medical Sciences,Shandong University,Jinan 250012,China
Shandong Key R & D Program FundingShandong Natural Science Foundation
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