Ganglioside sialidase distribution in mucolipidosis type IV cultured fibroblasts |
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| Authors: | M Zeigler G Bach |
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| Affiliation: | 1. School of Energy and Environment, Anhui University of Technology, Maanshan, Anhui 243002, China;2. State Key Laboratory of Clean Energy Utilization, Zhejiang University, Hangzhou, Zhejiang 310027, China;3. State Key Laboratory of Coal Combustion, School of Energy and Power Engineering, Huazhong University of Science and Technology, Wuhan 430074, China;1. State Key Laboratory Base of Eco-chemical Engineering, College of Chemistry and Molecular Engineering, Qingdao University of Science and Technology, Qingdao 266042, China;2. School of Medicine and Pharmacy, Ocean University of China, Qingdao 266003, China |
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| Abstract: | The subcellular distribution of ganglioside sialidase in Mucolipidosis IV (ML IV) cells was characterized by a series of Percoll gradients. Similar to normal cells, the enzyme cosedimented with plasma membrane markers, although this activity was reduced and exhibited decreased solubility in ML IV cells. Only trace amounts of ganglioside sialidase (less than 5%) was found in the lysosomes of normal cells. This activity was apparently reduced in ML IV cells but its minute activity in controls excluded further characterization of these differences. Plasma membranes on 6.7 and 5.6% Percoll gradients were biomodally distributed. Ganglioside sialidase in normal cells was found to be in both the heavier and the lighter membrane fractions, whereas the enzyme in ML IV cells was associated mainly with the denser membrane fraction. These data indicate that the enzyme in ML IV cells is characteristically different from normal in that it exhibits reduced activity and solubility and a different plasma membrane distribution. |
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