Dysregulated expression and distribution of Kif5αin neurites of wobbler motor neurons  

作　　者：Kilian Kürten Anne-Christin Gude Aimo Samuel Christian Epplen Jan Stein Carsten Theiss Veronika Matschke 

机构地区：[1]Department of Cytology,Institute of Anatomy,Medical Faculty,Ruhr University Bochum,Bochum,Germany

出　　处：《Neural Regeneration Research》2023年第1期150-154,共5页中国神经再生研究（英文版）

基　　金：supported by FoRUM–F976-20 (Ruhr-University Bochum)(to VM and CT)

摘　　要：Impaired axonal transport has been observed in patients with amyotrophic lateral sclerosis(ALS)and in animal models,suggesting that transport proteins likely play a critical role in the pathological mechanism of ALS.Dysregulation of Kinesin-family-member 5α(Kif5α),a neuron-specific isoform of heavy chain kinesin family,has been described in several neurological disorders,in humans and animal models,including ALS.In this study,we determined Kif5αexpression by gene sequencing,quantitative reverse transcription-polymerase chain reaction,and western blot assay in the cervical spinal cord of wobbler mice and immunofluorescence staining in dissociated cultures of the ventral horn.Further,we observed the distribution of Kif5αand mitochondria along motor neuronal branches by confocal imaging.Our results showed that Kif5αexpression was greatly dysregulated in wobbler mice,which resulted in altered distribution of Kif5αalong motor neuronal branches with an abnormal mitochondrial distribution.Thus,our results indicate that dysregulation of Kif5 and therefore abnormal transport in motor neuronal branches in this ALS model could be causative for several pathological findings at the cellular level,like misallocation of cytoskeletal proteins or organelles like mitochondria.

关 键 词：amyotrophic lateral sclerosis cell culture KINESIN MITOCHONDRIA NEURODEGENERATION spinal cord TRANSPORT 

分 类 号：R744.8[医药卫生—神经病学与精神病学]