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Int. J. Mol. Sci. 2016, 17(11), 1820; doi:10.3390/ijms17111820

Expression of Carbonic Anhydrase I in Motor Neurons and Alterations in ALS

1
Department of Biological Sciences, Xi’an Jiaotong-Liverpool University, Suzhou 215123, China
2
Department of Neurobiology, Barrow Neurological Institute, Phoenix, AZ 85013, USA
*
Author to whom correspondence should be addressed.
Academic Editors: Terrence Piva and Kurt A. Jellinger
Received: 9 August 2016 / Revised: 20 October 2016 / Accepted: 24 October 2016 / Published: 1 November 2016
(This article belongs to the Section Molecular Pathology, Diagnostics, and Therapeutics)
View Full-Text   |   Download PDF [4709 KB, uploaded 1 November 2016]   |  

Abstract

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. View Full-Text
Keywords: carbonic anhydrase 1 (CA1); amyotrophic lateral sclerosis (ALS); endoplasmic reticulum (ER); motor neuron; apoptosis carbonic anhydrase 1 (CA1); amyotrophic lateral sclerosis (ALS); endoplasmic reticulum (ER); motor neuron; apoptosis
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Liu, X.; Lu, D.; Bowser, R.; Liu, J. Expression of Carbonic Anhydrase I in Motor Neurons and Alterations in ALS. Int. J. Mol. Sci. 2016, 17, 1820.

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