Phosphatidylserine improves axonal transport by inhibition of HDAC and has potential in treatment of neurodegenerative diseases

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英文篇名：Phosphatidylserine improves axonal transport by inhibition of HDAC and has potential in treatment of neurodegenerative diseases 作者：Shiran ; Naftelberg ; Gil ; Ast ; Eran ; Perlson 英文作者：Shiran Naftelberg;Gil Ast;Eran Perlson;Department of Human Molecular Genetics and Biochemistry,Sackler Faculty of Medicine,Tel Aviv University;Department of Physiology and Pharmacology,Sackler Faculty of Medicine,Tel Aviv University; 英文关键词：axonal transport;;neurodegeneration;;microtubule;;familial dysautonomia;;phosphatidylserine;;HDAC6 中文刊名：SJZY 英文刊名：中国神经再生研究(英文版) 机构：Department of Human Molecular Genetics and Biochemistry,Sackler Faculty of Medicine,Tel Aviv University;Department of Physiology and Pharmacology,Sackler Faculty of Medicine,Tel Aviv University; 出版日期：2017-04-15 出版单位：Neural Regeneration Research 年：2017 期：v.12 基金：provided by grants from the Dysautonomia Foundation.Israel Science Foundation(ISF)[142/13,1439/14];; by Teva Pharmaceutical Industries Ltd as part of the Israeli National Network of Excellence in Neuroscience(NNE)[1234944];; supported by grants from the Israel Science Foundation(ISF)[561/11];; the European Research Council(ERC)[309377];; supported by grants from Teva Pharmaceutical Industries Ltd.under the Israeli National Network of Excellence in Neuroscience 语种：英文; 页：SJZY201704006 页数：4 CN：04 ISSN：11-5422/R 分类号：32-35

摘要

Familial dysautonomia(FD) is a rare children neurodegenerative disease caused due to a point mutation in the IKBKAP gene that results in decreased IKK complex-associated protein(IKAP) protein production. The disease affects mostly the dorsal root ganglion(DRG) and the sympathetic ganglion. Recently, we found that the molecular mechanisms underlying neurodegeneration in FD patients are defects in axonal transport of nerve growth factors and microtubule stability in the DRG. Neurons are highly polarized cells with very long axons. In order to survive and maintain proper function, neurons depend on transport of proteins and other cellular components from the neuronal body along the axons. We further demonstrated that IKAP is necessary for axon maintenance and showed that phosphatidylserine acts as an HDAC6 inhibitor to rescue neuronal function in FD cells. In this review, we will highlight our latest research findings.
        Familial dysautonomia(FD) is a rare children neurodegenerative disease caused due to a point mutation in the IKBKAP gene that results in decreased IKK complex-associated protein(IKAP) protein production. The disease affects mostly the dorsal root ganglion(DRG) and the sympathetic ganglion. Recently, we found that the molecular mechanisms underlying neurodegeneration in FD patients are defects in axonal transport of nerve growth factors and microtubule stability in the DRG. Neurons are highly polarized cells with very long axons. In order to survive and maintain proper function, neurons depend on transport of proteins and other cellular components from the neuronal body along the axons. We further demonstrated that IKAP is necessary for axon maintenance and showed that phosphatidylserine acts as an HDAC6 inhibitor to rescue neuronal function in FD cells. In this review, we will highlight our latest research findings.

引文

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