title: Mitochondrial dynamic abnormalities in amyotrophic lateral sclerosis.
abstract: Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease characterized by progressive loss of motor neurons in the brainstem and spinal cord. Currently, there is no cure or effective treatment for ALS and the cause of disease is unknown in the majority of ALS cases. Neuronal mitochondria dysfunction is one of the earliest features of ALS. Mitochondria are highly dynamic organelles that undergo continuous fission, fusion, trafficking and turnover, all of which contribute to the maintenance of mitochondrial function. Abnormal mitochondrial dynamics have been repeatedly reported in ALS and increasing evidence suggests altered mitochondrial dynamics as possible pathomechanisms underlying mitochondrial dysfunction in ALS. Here, we provide an overview of mitochondrial dysfunction and dynamic abnormalities observed in ALS, and discuss the possibility of targeting mitochondrial dynamics as a novel therapeutic approach for ALS.
authors.affiliation: Department of Pathology, Case Western Reserve University, Cleveland, OH USA.
authors.forename: Zhen
authors.initials: Z
authors.lastname: Jiang
authors.affiliation: Department of Pathology, Case Western Reserve University, Cleveland, OH USA.
authors.forename: Wenzhang
authors.initials: W
authors.lastname: Wang
authors.affiliation: College of Sciences, University of Texas at San Antonio, San Antonio, TX USA.
authors.forename: George
authors.initials: G
authors.lastname: Perry
authors.affiliation: Department of Pathology, Case Western Reserve University, Cleveland, OH USA.
authors.forename: Xiongwei
authors.initials: X
authors.lastname: Zhu
authors.affiliation: Department of Pathology, Case Western Reserve University, Cleveland, OH USA.
authors.forename: Xinglong
authors.initials: X
authors.lastname: Wang
keywords: ALS; Mitochondrial biogenesis and mitophagy; Mitochondrial dynamics; Mitochondrial dysfunction; Mitochondrial fission and fusion; Mitochondrial trafficking