Liu Xiaochen, Lu Deyi, Bowser Robert, Liu Jian
Department of Biological Sciences, Xi'an Jiaotong-Liverpool University, Suzhou 215123, China.
Department of Neurobiology, Barrow Neurological Institute, Phoenix, AZ 85013, USA.
Int J Mol Sci. 2016 Nov 1;17(11):1820. doi: 10.3390/ijms17111820.
Carbonic anhydrase I (CA1) is the cytosolic isoform of mammalian α-CA family members which are responsible for maintaining pH homeostasis in the physiology and pathology of organisms. A subset of CA isoforms are known to be expressed and function in the central nervous system (CNS). CA1 has not been extensively characterized in the CNS. In this study, we demonstrate that CA1 is expressed in the motor neurons in human spinal cord. Unexpectedly, a subpopulation of CA1 appears to be associated with endoplasmic reticulum (ER) membranes. In addition, the membrane-associated CA1s are preferentially upregulated in amyotrophic lateral sclerosis (ALS) and exhibit altered distribution in motor neurons. Furthermore, long-term expression of CA1 in mammalian cells activates apoptosis. Our results suggest a previously unknown role for CA1 function in the CNS and its potential involvement in motor neuron degeneration in ALS.
碳酸酐酶I（CA1）是哺乳动物α-碳酸酐酶家族成员的胞质异构体，负责在生物体的生理和病理过程中维持pH稳态。已知一部分碳酸酐酶异构体在中枢神经系统（CNS）中表达并发挥作用。CA1在中枢神经系统中尚未得到广泛研究。在本研究中，我们证明CA1在人类脊髓的运动神经元中表达。出乎意料的是，一部分CA1似乎与内质网（ER）膜相关。此外，膜相关的CA1在肌萎缩侧索硬化症（ALS）中优先上调，并在运动神经元中表现出分布改变。此外，CA1在哺乳动物细胞中的长期表达会激活细胞凋亡。我们的结果表明CA1在中枢神经系统中的功能有一个以前未知的作用，并且它可能参与了ALS中运动神经元的退化。
