Ngo S T, Steyn F J
Queensland Brain Institute, The University of Queensland, St Lucia, Brisbane 4072 Australia ; School of Biomedical Sciences, The University of Queensland, St Lucia, Brisbane 4072 Australia ; Department of Neurology, Royal Brisbane and Women's Hospital, Herston, Brisbane 4006 Australia ; University of Queensland Centre for Clinical Research, The University of Queensland, Herston, Brisbane, 4029 Australia.
School of Biomedical Sciences, The University of Queensland, St Lucia, Brisbane 4072 Australia ; University of Queensland Centre for Clinical Research, The University of Queensland, Herston, Brisbane, 4029 Australia.
Cell Regen. 2015 Aug 27;4(1):5. doi: 10.1186/s13619-015-0019-6. eCollection 2015.
Amyotrophic lateral sclerosis (ALS) is a fatal, neurodegenerative disease that is characterized by the selective degeneration of upper motor neurons and lower spinal motor neurons, resulting in the progressive paralysis of all voluntary muscles. Approximately 10 % of ALS cases are linked to known genetic mutations, with the remaining 90 % of cases being sporadic. While the primary pathology in ALS is the selective death of upper and lower motor neurons, numerous studies indicate that an imbalance in whole body and/or cellular metabolism influences the rate of progression of disease. This review summarizes current research surrounding the impact of impaired metabolic physiology in ALS. We extend ideas to consider prospects that lie ahead in terms of how metabolic alterations may impact the selective degeneration of neurons in ALS and how targeting of adenosine triphosphate-sensitive potassium (KATP) channels may represent a promising approach for obtaining neuroprotection in ALS.
肌萎缩侧索硬化症（ALS）是一种致命的神经退行性疾病，其特征是上运动神经元和脊髓下运动神经元选择性退化，导致所有随意肌进行性麻痹。约10%的ALS病例与已知基因突变有关，其余90%的病例为散发性。虽然ALS的主要病理是上、下运动神经元的选择性死亡，但大量研究表明，全身和/或细胞代谢失衡会影响疾病的进展速度。本综述总结了目前围绕ALS中代谢生理受损影响的研究。我们拓展思路，考虑代谢改变如何影响ALS中神经元的选择性退化，以及靶向三磷酸腺苷敏感性钾（KATP）通道如何可能成为在ALS中获得神经保护的一种有前景的方法等方面的未来前景。
