Affiliation:
1. Graduate School of Medicine, Chiba University, Chiba, Japan
Abstract
Molecular protein motors play key roles in processes such as intracellular cargo transport and brain wiring, and failure of function can give rise to serious diseases. Kinesin-1, a member of the kinesin superfamily (also known as KIFs) is a two-headed motor protein that uses energy derived from ATP hydrolysis to transport diverse types of intracellular cargo toward the plus-ends of microtubules within axons. Recent studies at the level of a single molecule have provided extensive knowledge on how kinesin-1 moves along microtubules. Further elucidation of kinesin-1 movement may shed light on its influence on axon generation, thereby leading to therapies for diseases such as spastic paraplegia type 10 (SPG10), the subject of this review. SPG10 is an autosomal dominant form of hereditary spastic paraplegia caused by mutations in KIF5A, which encodes one of the isoforms of kinesin-1 (KIF5A, KIF5B, and KIF5C). Although little is known about the cargo of KIF5A, a recent study revealed an axonal transport defect of mitochondria in a KIF5A − / − mouse model. This review discusses the consensus moving model of kinesin-1 and the pathogenicity of SPG10 caused by defective KIF5A function.
Subject
Neurology (clinical),General Neuroscience
Cited by
21 articles.
订阅此论文施引文献
订阅此论文施引文献,注册后可以免费订阅5篇论文的施引文献,订阅后可以查看论文全部施引文献