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NEUROLOGY 1980;30:719
© 1980 American Academy of Neurology

Carbonic anhydrase and 2', 3' cyclic nucleotide 3'-phosphohydrolase activity in normal human brain and in demyelinating diseases

Marjorie B. Lees, Ph.D., Victor S. Sapirstein, Ph.D., David S. Reiss, Ph.D. and Edwin H. Kolodny, M.D.

Biochemistry Department, Eunice Kennedy Shriver Center for Mental Retardation, Waltham, and the Departments of BiologicaI Chemistry (Drs. Lees, Sapirstein, and Reiss) and Neurology (Dr. Kolodny), Harvard Medical School, Boston, MA.

The activities of carbonic anhydrase and 2', 3' cyclic nucleotide 3'-phosphohydrolase (CNPase) were measured in gray and white matter and in myelin from human brains obtained at autopsy. Carbonic anhydrase activity increased with age, and at all ages a major part of the activity was associated with membrane fractions. The percentage of membrane-bound carbonic anhydrase was lower than normal in white matter from Krabbe disease and adrenoleukodystrophy; isolated myelin had a low specific activity in these diseases. CNPase activity was decreased in both white matter and myelin, but was somewhat higher than normal in gray matter. Although the yield of myelin from a case of metachromatic leukodystrophy was markedly reduced, changes in the enzyme activities were minimal.

Address correspondence and reprint requests to Dr. Lees, Eunice Kennedy Shriver Center for Mental Retardation, 200 Trapelo Road, Waltham, MA 02154.

Accepted for publication October 9, 1979.

This work was supported in part by US. Public Health Service Grants Nos. NS 13649, NS 13710, HD 05515, and HD 04147.






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