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Objective Carbonic anhydrase Ⅰ (CA Ⅰ) protein levels were found to be significantly increased in the spinal cords of both mutant SOD 1-linked and sporadic ALS patients.CA Ⅰ is the cytoplamic isoform of the CA family which comprises of a total of 16 members.It is not known how change of CA Ⅰ might be related to ALS pathogenesis.The aims of this study are: (1) determine whether CA Ⅰ is expressed in motor neurons in the spinal cord; (2) determine how is this expression is affected by ALS at the cellular level; (3) explore the mechanism by which CA Ⅰ might play in ALS pathology.Methods Immunohistochemistry and fluorescent immunostairming techniques were used to determine the pattern and changes of CA Ⅰ expression in human spinal cords.Protein biochemistry and Western Blot analysis were used to examine the potential mechanism of subcellular location of CA Ⅰ.Results (1) CA Ⅰ is expressed in the motor neurons in human spinal cord; (2) a subpopulation of CA Ⅰ is associated with ER membranes; (3) CA Ⅰ change in ALS is more enhanced with membrane-associated CA Ⅰ.Conclusion CA Ⅰ might play a role in ALS pathology via ER stress-mediated pathway.