Deficiency of Galactosidase Activity in Multiple Organs in GM2-Deficient Mice

• Published in: Journal of Clinical Biochemistry and Nutrition Vol. 13; no. 3; pp. 189 - 197
• Main Authors: AOYAGI, Takaaki, OSANAI, Taka, WADA, Takao, KOJIMA, Fukiko, NAGAI, Machiko, HARADA, Shigeko, TAKEUCHI, Tomio, SUZUKI, Akemi, NAGAI, Yoshitaka
• Format: Journal Article
• Language: English
• Published: Tokyo SOCIETY FOR FREE RADICAL RESEARCH JAPAN 1992; Institute of Applied Biochemistry; Japan Science and Technology Agency
• Subjects: Biological and medical sciences; Errors of metabolism; gangliosides; glycosidases; GM2 deficiency; Lipids (lysosomal enzyme disorders, storage diseases); Medical sciences; Metabolic diseases; proteases; WHT/Ht mouse; Animal model; Gangliosidosis; Enzyme; Deficiency; Rodentia; Galactosidase; Metabolic diseases; Sphingolipidosis; Enzymopathy; Genetic disease; Vertebrata; Mammalia; Enzymatic activity; Mouse; Organ
• ISSN: 0912-0009; 1880-5086
• DOI: 10.3164/jcbn.13.189

WHT/Ht mice are known to have GM2 deficiency especially in the liver. In order to know the relationship of this abnormality to the general metabolism in organs or cells, we compared 17 hydrolytic enzyme activities in the liver, brain, spleen, and kidney between the WHT/Ht mice and control BALB/c mice or without administration of GM2 and GM3. Regardless of the GM2 or GM3 treatment, the galactosidase activity was markedly low in all of the tested organs in the WHT/Ht mice when compared with that in the control animals. This consistency stands in contrast with the known organ-dependent heterogeneity of ganglioside expression and thus indicates independency of the GM2 deficiency from the galactosidase deficiency in this model animal.