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| 1 | 核质转运异常与神经退行性疾病显示文摘核质转运过程作为真核细胞的基本生命活动之一,对细胞生理和病理过程都至关重要。大量证据表明,核质转运异常是神经退行性疾病的关键致病因素,而核质转运障碍的调控机制对于阐明神经退行性疾病的发病机制和干预治疗至关重要。本文搜集20年来核质转运异常在神经退行性疾病发病机制中的相关研究,综述在肌萎缩脊髓侧索硬化症/前额颞叶痴呆、亨廷顿舞蹈症和脊髓小脑共济失调等神经退行性疾病中的核质转运现象和涉及的调控机制,进一步探讨在目前研究中存在的问题并提出建议,为神经退行性疾病的致病机制和药物靶标研究提供理论依据。 | 王子见 李子恒 常亦昆 张甜 任媛媛 何凤琴 | 2021 | 药学学报2021，56，5： | 1 |
| 2 | The importance of fasciculation and elongation protein zeta-1 in neural circuit establishment and neurological disorders显示文摘The human brain contains an estimated 100 billion neurons that must be systematically organized into functional neural circuits for it to function properly.These circuits range from short-range local signaling networks between neighboring neurons to long-range networks formed between various brain regions.Compelling converging evidence indicates that alterations in neural circuits arising from abnormalities during early neuronal development or neurodegeneration contribute significantly to the etiology of neurological disorders.Supporting this notion,efforts to identify genetic causes of these disorders have uncovered an over-representation of genes encoding proteins involved in the processes of neuronal differentiation,maturation,synaptogenesis and synaptic function.Fasciculation and elongation protein zeta-1,a Kinesin-1 adapter,has emerged as a key central player involved in many of these processes.Fasciculation and elongation protein zeta-1-dependent transport of synaptic cargoes and mitochondria is essential for neuronal development and synapse establishment.Furthermore,it acts downstream of guidance cue pathways to regulate axo-dendritic development.Significantly,perturbing its function causes abnormalities in neuronal development and synapse formation both in the brain as well as the peripheral nervous system.Mutations and deletions of the fasciculation and elongation protein zeta-1 gene are linked to neurodevelopmental disorders.Moreover,altered phosphorylation of the protein contributes to neurodegenerative disorders.Together,these findings strongly implicate the importance of fasciculation and elongation protein zeta-1 in the establishment of neuronal circuits and its maintenance. | Rafhanah Banu Bte Abdul Razar Yinghua Qu Saravanan Gunaseelan John Jia En Chua | 2022 | Neural Regeneration Research2022，17，6： | 0 |
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