TY - JOUR
T1 - Expression of carbonic anhydrase I in motor neurons and alterations in ALS
AU - Liu, Xiaochen
AU - Lu, Deyi
AU - Bowser, Robert
AU - Liu, Jian
N1 - Publisher Copyright:
© 2016 by the authors.
PY - 2016/11
Y1 - 2016/11
N2 - 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.
AB - 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.
KW - Amyotrophic lateral sclerosis (ALS)
KW - Apoptosis
KW - Carbonic anhydrase 1 (CA1)
KW - Endoplasmic reticulum (ER)
KW - Motor neuron
UR - http://www.scopus.com/inward/record.url?scp=84994528978&partnerID=8YFLogxK
U2 - 10.3390/ijms17111820
DO - 10.3390/ijms17111820
M3 - Article
C2 - 27809276
AN - SCOPUS:84994528978
SN - 1661-6596
VL - 17
JO - International Journal of Molecular Sciences
JF - International Journal of Molecular Sciences
IS - 11
M1 - 1820
ER -